OBJECTIVE: To analyze clinical manifestations, diagnosis and treatment of annular pancreas in neonates. METHODS: Clinical data of 114 neonates with annular pancreas admitted in the Children's Hospital of Zhejiang University from January 2009 to December 2018 were reviewed. The demographic parameters (gestational age, birth weight), clinical manifestations, onset time, results of antenatal examination, associated anomalies, radiological findings, operations, postoperative complications were analyzed. RESULTS: One hundred and two cases were examined by abdominal echography, in which 68 cases showed duodenal obstruction, 4 cases showed annular pancreas. Plain abdomen X-ray examination performed in 113 cases before operation, 76 cases presented double-bubble sign, 12 cases presented single-bubble sign and 5 cases had high-position intestinal obstruction. Upper gastrointestinal radiography (UGI) was performed in 103 cases, which suggested duodenal obstruction in 102 cases. Operations were performed in all cases, of which 69 cases were operated under laparoscopy including 1 case converted to open laparotomy. The mean fasting time after surgery was (7.8±2.7) d, and the mean length of hospital stay was (16.9±10.1) d. Five patients had postoperative complications. The incidence of postoperative complications in antenatal abnormal group was lower than that in the antenatal non-abnormal group (<0.05); the average fasting time in laparoendscopic surgery group was shorter than that in traditional laparotomy group (<0.05). CONCLUSIONS Neonates with recurrent vomiting early after birth should be highly suspected to have annular pancreas. The fetal chromosome examination should be performed with abnormal antenatal screening. Surgery is the only effective way to diagnose and treat annular pancreas, and laparoscopic surgery could be the first choice for experienced doctors.
Duodenal Obstruction ; diagnostic imaging ; surgery ; Humans ; Infant, Newborn ; Laparoscopy ; Pancreas ; abnormalities ; diagnostic imaging ; pathology ; surgery ; Pancreatic Diseases ; diagnostic imaging ; pathology ; surgery ; Retrospective Studies

STUDY DESIGN: To induce scoliosis in young female Wistar rats using a noninvasive method and to validate this model. PURPOSE: To induce scoliosis in a rat model noninvasively by bracing and to study the corresponding gene-expression profile in the spine and different organs. OVERVIEW OF LITERATURE: Scoliosis involves abnormal lateral curvature of the spine, the causes of which remain unclear. In the literature, it is suggested that scoliosis is genetically heterogeneous, as there are multiple factors involved directly or indirectly in its pathogenesis. Clinical and experimental studies were conducted to understand the etiology of anatomical alterations in the spine and internal organs, as the findings could help clinicians to establish new treatment approaches. METHODS: Twelve female Wistar rats aged 21 days were chosen for this study. Customized braces and real-time polymerase chain reaction (RT-PCR) primers for rats were designed using Primer 3 software. Radiological analysis (X-rays), histopathological studies, SYBR green, and RT-PCR analysis were performed. RESULTS: The spines of six rats were braced in a deformed position, which resulted in a permanent structural deformity as confirmed by X-ray studies. The remaining rats were used as controls. Quantitative studies of the expression of various genes (osteocalcin, pleiotrophins, matrix metalloproteinase-2 [MMP2] and MMP9, TIMP, interleukins 1 and 6, tumor necrosis factor-α) showed their differential expression and significant upregulation (p < 0.05) in different organs of scoliotic rats in comparison to those in control rats. Histopathological findings showed tissue necrosis and fibrosis in the brain, retina, pancreas, kidney, liver, and disc of scoliotic rats. CONCLUSIONS: Bracing is a noninvasive method for inducing scoliosis in an animal model with 100% reliability and with corresponding changes in gene expression. Scoliosis does not just involve a spine deformity, but can be referred to as a systemic disease on the basis of the pathological changes observed in various internal organs.
Animals ; Braces ; Brain ; Congenital Abnormalities ; Female ; Fibrosis ; Gene Expression ; Humans ; Inflammation ; Interleukins ; Kidney ; Liver ; Matrix Metalloproteinase 2 ; Methods ; Models, Animal ; Necrosis ; Pancreas ; Rats ; Rats, Wistar ; Real-Time Polymerase Chain Reaction ; Retina ; Scoliosis ; Spine ; Up-Regulation

Heterotaxy syndrome (HS) is a congenital disorder resulting from an abnormal arrangement of visceral organs across the normal left-right axis in the embryonic period. HS is usually associated with multiple anomalies, including defects of the major cardiovascular system and the extracardiovascular system such as intestinal malrotation, abnormal lung lobulation, bronchus anomalies, and pancreatic dysplasia. Although pancreatic dysplasia is occasionally accompanied with HS, the occurrence of diabetes mellitus (DM) due to pancreatic dysplasia in HS is rarely reported. We here report a case involving 13-year-old girl with DM caused by agenesis of the dorsal pancreas and HS diagnosed on the basis of the presence of a double-outlet right ventricle with bilateral pulmonary stenosis and intestinal malrotation with duodenal cyst. Timely diagnosis and treatment with insulin improved glycemic control.
Adolescent ; Bronchi ; Cardiovascular System ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Diabetes Mellitus* ; Diagnosis ; Double Outlet Right Ventricle ; Female ; Heterotaxy Syndrome* ; Humans ; Insulin ; Lung ; Pancreas* ; Pulmonary Valve Stenosis

This pictorial review aims to illustrate the magnetic resonance imaging (MRI) findings and presentation patterns of anatomical variations and various benign and malignant pathologies of the duodenum, including sphincter contraction, major papilla variation, prominent papilla, diverticulum, annular pancreas, duplication cysts, choledochocele, duodenal wall thickening secondary to acute pancreatitis, postbulbar stenosis, celiac disease, fistula, choledochoduodenostomy, external compression, polyps, Peutz-Jeghers syndrome, ampullary carcinoma and adenocarcinoma. MRI is a useful imaging tool for demonstrating duodenal pathology and its anatomic relationships with adjacent organs, which is critical for establishing correct diagnosis and planning appropriate treatment, especially for surgery.
Ampulla of Vater/anatomy & histology/radiography ; Choledochal Cyst/pathology/radiography ; Diverticulum/radiography ; Duodenal Diseases/pathology/*radiography ; Duodenum/*anatomy & histology/radiography ; Humans ; *Magnetic Resonance Imaging ; Pancreas/abnormalities/anatomy & histology/radiography ; Pancreatic Diseases/radiography

OBJECTIVETo explore the value and safety of the application of enteral nutrition in pediatric surgery.

METHODSClinical data of 56 children patients who could not undertake oral feeding or those who required enteral nutrition by nasojejunal tube over 7 days in our ward from June 2007 to May 2013 were retrospectively analyzed. Children younger than one year old received enteral nutritional formulation Ai Er Shu mainly composed of short peptides or amino acids, and children over one year received Small peptide or Nengquansu mainly composed of short peptide.

RESULTSAmong these 56 children patients, primary disease was pancreatitis in 20 cases, lacerated wound in 10, duodenal perforation in 6, duodenal septum-shape stenosis in 9, annular pancreas in 6, and hepatoblastoma in 5. All the patients successfully completed the enteral nutrition therapy within 7-37 (18±9.9) days with good recovery. During the treatment, 5 cases (8.9%) developed diarrhea, 3 cases (5.4%) nausea and vomiting, 2 cases (3.6%) mild abdominal distension, 5 cases(8.9%) mild abdominal pain, and the symptoms were relieved after adjustment of nutrient solution concentration and infusion rate. After enteral nutrition for 10 days, the nutritional status of children was improved, and weight, blood red cell count, hemoglobin, serum albumin, prealbumin increased significantly (P<0.05).

CONCLUSIONApplication of enteral nutrition in pediatric surgery is safe and effective, which can improve the nutritional status of children and promote the recovery.

Abdominal Pain ; Child ; Diarrhea ; Enteral Nutrition ; Humans ; Infant ; Nutritional Status ; Pancreas ; abnormalities ; Pancreatic Diseases ; Pancreatitis ; Retrospective Studies

Though congenital anomalies of the pancreas and pancreatic duct are relatively uncommon and they are often discovered as an incidental finding in asymptomatic patients, some of these anomalies may lead to various clinical symptoms such as recurrent abdominal pain, nausea and vomiting. Recognition of these anomalies is important because these anomalies may be a surgically correctable cause of recurrent pancreatitis or the cause of gastric outlet obstruction. An awareness of these anomalies may help in surgical planning and prevent inadvertent ductal injury. The purpose of this article is to review normal pancreatic embryology, the appearance of ductal anatomic variants and developmental anomalies of the pancreas, with emphasis on magnetic resonance cholangiopancreaticography and multidetector computed tomography.
Cholangiopancreatography, Magnetic Resonance/*methods ; Humans ; Multidetector Computed Tomography/*methods ; Pancreas/abnormalities ; Pancreatic Diseases/congenital/pathology/radiography ; Pancreatic Ducts/*abnormalities

Portal annular pancreas is one of the pancreatic fusion anomalies in which the uncinate process of the pancreas extends to fuse with the dorsal pancreas by encircling the portal vein or superior mesenteric vein. We report two consecutive patients with portal annular pancreas. The first case is a 71-year-old male patient who underwent a pancreaticoduodenectomy for intraductal papillary mucinous neoplasm in the head of pancreas. His preoperative computed tomography scan showed the suprasplenic type portal annular pancreas. The second case is a 74-year-old female patient who underwent a laparoscopic anterior radical antegrade modular pancreatosplenectomy (RAMPS) for pancreatic body cancer. In operative finding, portal confluence (superior mesenteric vein-splenic vein-portal vein) was encased with the uncinate process of pancreas in both cases. Therefore, they required pancreatic division at the pancreatic neck portion twice. During the postoperative period, grade B and A, respectively, postoperative pancreatic fistulas occurred and were controlled by conservative management. Surgeons need to know about this rare pancreatic condition prior to surgical intervention to avoid complications, and to provide patients with well-designed, case-specific pancreatic surgery.
Adenocarcinoma, Mucinous/diagnosis/surgery ; Aged ; Female ; Humans ; Male ; Mesenteric Veins/radiography ; Pancreas/abnormalities ; Pancreatic Diseases/*diagnosis/therapy ; Pancreatic Fistula/etiology ; Pancreatic Neoplasms/diagnosis/surgery ; Pancreaticoduodenectomy/adverse effects ; Portal Vein/radiography ; Splenic Vein/radiography ; Tomography, X-Ray Computed

A 49-year-old female was admitted to our hospital for acute pancreatitis. The abdomen CT scan incidentally showed midline liver with hepatomegaly, centrally located gallbladder, pancreas truncation, right sided small bowel, left sided large bowel, interruption of the inferior vena cava with azygos continuation, preduodenal portal vein, and multiple spleens in the left upper quadrant. In MRCP, the head of pancreas was enlarged and short main pancreatic duct without accessory duct was showed. EUS revealed enlarged ventral pancreas with a main pancreatic duct of normal caliber, absence of the accessory pancreatic duct and the dorsal pancreas. She was diagnosed as polysplenia syndrome with agenesis of dorsal pancreas. It is a rare congenital anomaly frequently associated with various visceral anomalies including multiple spleens, impaired visceral lateralization, congenital heart diseases, gastrointestinal abnormalities and azygos continuation of the inferior vena cava. We report a case of polysplenia syndrome with agenesis of dorsal pancreas presenting acute pancreatitis.
Acute Disease ; Congenital Abnormalities/*diagnosis/ultrasonography ; Endosonography ; Female ; Heterotaxy Syndrome/*diagnosis/ultrasonography ; Humans ; Magnetic Resonance Imaging ; Middle Aged ; Pancreas/abnormalities/ultrasonography ; Pancreatitis/*diagnosis ; Tomography, X-Ray Computed

Agenesis of the dorsal pancreas is a rare congenital anomaly that arises from the failure of the dorsal pancreatic bud of endodermal cells to form the body and tail of the pancreas and can manifest as diabetes. A 24-year-old man, who had been treated with insulin for 7 years, presented with epigastric pain, vomiting, and watery diarrhea. Abdominal computed tomography showed only the head of the pancreas without visualization of the pancreatic body and tail. Left renal agenesis and absence of the left vertebral pedicle in T12 were also observed. The duct of Santorini and the duct in the body and tail were not visible in magnetic resonance cholangiopancreatography. The associated anomalies reported here are very rare globally. We report a case of complete agenesis of the dorsal pancreas with multiple congenital abnormalities and diabetes mellitus.
Cholangiopancreatography, Magnetic Resonance ; Congenital Abnormalities ; Diabetes Mellitus ; Diarrhea ; Endoderm ; Head ; Humans ; Insulin ; Kidney ; Kidney Diseases ; Pancreas ; Pancreatic Ducts ; Vomiting ; Young Adult

The most common causes of acute pancreatitis are microlithiasis and alcohol. In pediatrics, anomalies in pancreaticobiliary system should be considered as possible causes. Among many anomalies, pancreas divisum associated with anomalous pancreaticobiliary ductal union (APBDU) is very rare. APBDU is associated with acute pancreatitis, choledochal cyst, and gallbladder cancer. Pancreas divisum is also a well known cause of acute recurrent pancreatitis. In adult cases with such conditions, the role of endoscopic management including sphincterotomy or stenting through the Santorini duct is well documented. However, it is still controversial to perform endoscopic retrograde cholangiopancreatography in pediatrics. Herein, we experienced a case of 4 year 7 month old female patient suffered from recurrent attacks of acute pancreatitis, which were caused by APBDU and incomplete pancreas divisum. She was treated by endoscopic sphincteretomy of both openings to the Santorini's and Wirsung's ducts. Thus, we report this interesting case with literature review.
Abnormalities, Multiple ; Acute Disease ; Child, Preschool ; Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct/*abnormalities/surgery ; Female ; Humans ; Pancreas/*abnormalities ; Pancreatic Ducts/*abnormalities/surgery ; Pancreatitis/*diagnosis/etiology/surgery ; Sphincterotomy, Endoscopic ; Tomography, X-Ray Computed