Mixed acinar-neuroendocrine carcinoma (MANEC) is a malignant pancreatic tumor that rarely occurs in children. It is diagnosed pathologically according to the proportion of neuroendocrine cells present, highlighting the need for surgical biopsy. A 13-year-old boy presented with a 10-cm palpable mass on CT. Surgical resection was performed, and the pathological diagnosis was MANEC. There were no postoperative complications, and the patient was discharged from the hospital 10 days after surgery. He is presently undergoing adjuvant chemotherapy. We reviewed historical MANEC cases published in the English literature. We concluded that pathological analysis of a surgically resected specimen is necessary for an accurate diagnosis of MANEC, and that publication of more cases is needed to determine the optimal management strategy for MANEC.
Adolescent ; Biopsy ; Carcinoma, Acinar Cell ; Carcinoma, Neuroendocrine ; Chemotherapy, Adjuvant ; Child ; Diagnosis ; Humans ; Immunohistochemistry ; Male ; Neuroendocrine Cells ; Postoperative Complications ; Publications ; Surgical Procedures, Operative

PURPOSE: The aim of this study was to analyze of the risk factors for surgical procedure on ileo-colic intussusception without leading point in children. METHODS: We retrospectively reviewed medical records of patient treated for ileo-colic intussusception between January 2003 and December 2014. We exclude the patients who had leading point. Because of the large difference on patient's numbers between non-operative group (cases of ileo-colic intussusceptions successfully reduced by air reduction) and operative group (cases underwent operation due to failed air reduction), we compared the data of operative group of patients without leading point between 2003 and 2014 with the data of non-operative group as control group from 2013 to 2014. Clinical features such as gender, age, body temperature, body weight in diagnosis, growth curves for age-gender-body weight, and laboratory data of blood test were compared. RESULTS: In non-operative group, total 94 patients who were treated successfully by the non-operative air reduction. In operative group, total 21 patients treated by surgical procedure. The age under 12 months, weight over upper 75 percentile group, increased segment neutrophil count, decreased hemoglobin level and lymphocyte count were significantly associated with a requirement for surgical procedure. CONCLUSION: We conclude that younger age, higher weight percentile group, increased segment neutrophil, decreased hemoglobin and lymphocyte are the independent risk factors related to operative treatment for ileo-colic intussusception in children. If primary air reduction is failed in patients with such risk factors, operative treatment over ultrasonography or secondary reduction can prevent unnecessary effort and complications, thus emphasizing the consideration of operative treatment when selecting treatment methods.
Body Temperature ; Body Weight ; Child* ; Diagnosis ; Hematologic Tests ; Humans ; Intussusception* ; Lymphocyte Count ; Lymphocytes ; Medical Records ; Neutrophils ; Retrospective Studies ; Risk Factors* ; Ultrasonography

Although Meckel's diverticulum is the most common vitellointestinal duct (VID) anomaly, patent vitellointestinal duct (PVID) is the most common symptomatic embryological defect. Patient may present with the anomaly itself or due to complications like intestinal obstruction secondary to volvulus, intussusception or adhesions. Prolapse occurs if the diverticulum is wide-mouthed enough to allow bowel to come out or due to increased intra-abdominal pressure like cry or cough. Bowel prolapse through PVID is rare and double prolapse of proximal as well as distal loop in a newborn is extremely rare. Omphalocele with prolapsing bowel through PVID as found in our index case is even rarer in literature. The pediatric surgeon should be familiar with these varied manifestations in the newborn because the prolapsed bowel can progress to gangrene and complications if not identified and operated upon early.
Cough ; Diverticulum ; Gangrene ; Hernia, Umbilical* ; Humans ; Ileum* ; Infant, Newborn ; Intestinal Obstruction ; Intestinal Volvulus ; Intussusception ; Meckel Diverticulum ; Prolapse* ; Vitelline Duct

Nodular hidradenoma was diagnosed in a 29-month-old girl on her axilla. Hidradenoma, sometimes designated as acrospiroma, is a benign sweat gland neoplasm, which mostly occurs in adults. Very few cases of hidradenoma have been documented in children in their first decade of life. This case demonstrates that when a child develops a skin nodule, nodular hidradenoma can be a diagnostic option.
Acrospiroma* ; Adult ; Axilla ; Child ; Child, Preschool* ; Female ; Humans ; Skin ; Sweat Gland Neoplasms

Abdominal actinomycosis is a rare and chronic progressive disease, especially in children. Clinically, it has non-specific symptoms and diagnostic findings as well as low prevalence, making it very difficult to diagnose prior to intraoperative pathological confirmation. For this reason, abdominal actinomycosis is commonly misdiagnosed as appendicitis. After the histopathological diagnosis of abdominal actinomycosis is made, patients should be administered an appropriate antibiotic such as penicillin. Here we describe a case of appendiceal actinomycosis in an 18-year-old girl who was initially diagnosed with acute appendicitis.
Actinomycosis* ; Adolescent ; Appendiceal Neoplasms ; Appendicitis* ; Child ; Diagnosis ; Female ; Humans ; Penicillins ; Prevalence

PURPOSE: Long time total parenteral nutrition (TPN) can induce irreversible liver damage. In this study, we investigated the associated factors of parenteral nutrition associated cholestasis (PNAC) in neonates. METHODS: We retrospectively reviewed 227 neonates (male:female=110:117) those who had received TPN over 2 weeks from March 2010 to February 2014. PNAC was defined as direct bilirubin was higher than 2.0 mg/dL without any cause except TPN. RESULTS: Overall incidence was 28.6%. PNAC was frequently developed in younger gestational age with lower birth weight. Episodes of sepsis, underlying bronchopulmonary dysplasia, history of necrotizing enterocolitis, and experience of gastrointestinal surgery increase the incidence of PNAC. PNAC was directly associated the duration of TPN and long period to full enteral feeding, reaching 60 and 150 mL/kg/day. Overall mortality rate was 9.7%. It was higher in PNAC group despite PNAC was not the primary cause of death. All survivors were recovered from cholestasis with encourage of enteral nutrition. CONCLUSION: PNAC in neonate was associated with younger gestational ages and lower birth weights, duration of TPN, or who experienced sepsis, necrotizing enterocolitis, gastrointestinal surgery or bronchopulmonary dysplasia.
Bilirubin ; Birth Weight ; Bronchopulmonary Dysplasia ; Cause of Death ; Cholestasis* ; Enteral Nutrition ; Enterocolitis, Necrotizing ; Gestational Age ; Humans ; Incidence ; Infant, Newborn* ; Liver ; Mortality ; Parenteral Nutrition* ; Parenteral Nutrition, Total ; Retrospective Studies ; Sepsis ; Survivors

The purpose of our study was to evaluate children who underwent hepatic resection for primary malignant hepatic tumor in the period from January 1994 to December 2001. A total of 8 patients, seven with hepatoblastoma (HB) and one with hepatocellular carcinoma (HCC). were studied. One HCC was respectable at the initial diagnosis, but five cases of unresectable HB received two cycles of transarterial chemoembolization (TACE) before operation. One patient with an unresectable HB with bone marrow metastasis was operated after one cycle of TACE and one cycle of systemic chemotherapy based on CCG-823F protocol. All 6 patients who underwent TACE and neoadjuvant chemotherapy showed marked redcuction in tumor volume and a clear outline of the lesion. Major complication was not noticed. Mean alpha-fetoprotei (alpha-FP) level at diagnosis, after neoadjuvant chemotherapy and after postoperative chemotherapy was 9,818 (42-35,350), 664, and 10.1 ng/mL, respectively. Half life of the alpha-FP after complete resection was 5.1 days (3.0-8.7 days). Median follow up period was 57.1 months (10-97 months) and all the patients are alive with NED. In conclusion, preoperative chemotherapy, especially TACE, is effective, safe, and useful to treat initially unresectable hepatoblastoma, and serial level of the serum alpha-FP is a useful tumor marker for diagnosis and monitoring therapeutic responses.
Bone Marrow ; Carcinoma, Hepatocellular ; Child ; Diagnosis ; Drug Therapy ; Follow-Up Studies ; Half-Life ; Hepatoblastoma ; Humans ; Neoplasm Metastasis ; Tumor Burden

This study reviews 14 years' experience of esophageal atresia with special emphasis on the clinical profile and the outcome. From May 1989 to February 2003, 65 cases of esophageal atresia (EA) were treated at Asan Medical Center. Boys outnumbered girls 2.4 to 1. Prematutity and low birth weight were 27.7% and 38.5%. Esophageal atresia with distal tracheoesophageal fistula (TEF) was the most common type (87.7%), followed by pure EA and H type fistula. Forty-six patients (70.8%) had one or more associated anomalies, cardiac malformations were the most common. Duodenal atresia was found in 7 cases. There were 6 patients (9.2%) with VATER cluster. VACTERL cluster was present in 18 patients (27.7%), one of who fulfilled the complete syndrome. Waterston group A, B and C made up 21.5%, 40.0% and 38.5% of the total group. Surgical treatment was attempted in 63 patients and deferred in 2 who had severe associated malformations. For EA with distal TEF, primary esophago-esophagostomy was carried out in 51 cases, and division of TEF and gastrostomy in 4 cases and no operation in 2 cases. For pure EA, colonic graft was done in 2 after gastrostomy and esophagostomy, and esophago-esophagostomy was performed in 2 after gastrostomy. Two TEF was carried out in 2 cases with H type TEF. The overall survival rate was 76.9%, and survival by Waterstuon classification was 100% in group A, 80.8% in B and 60.0% in C. Thorough workup for associated anomalies, interdepartmental approach and more careful surgical decision and technique are required to improve the outcome of EA.
Chungcheongnam-do ; Classification ; Colon ; Esophageal Atresia* ; Esophagostomy ; Female ; Fistula ; Gastrostomy ; Humans ; Infant, Low Birth Weight ; Infant, Newborn ; Survival Rate ; Tracheoesophageal Fistula ; Transplants

Preauricular sinus and preauricular skin tag are common childhood congenital anomalies. It is important for the pediatric surgeon to be familiar with the embryology and differentiation of head and neck structure to accurately diagnose and treat these lesions. Seventy-eight patients with preauricular sinus and twenty-eight with preauricular skin tag treated in the Department of pediatric surgery at Hanyang University Hospital from January 1981 to May 2002 were reviewed to determine relative frequency, clinical classification and appropriate treatment. The male to female ratio of preauricular sinus was 1:1.2, and preauricular skin tag was 1:1. The most commonly presenting age of sinus and skin tag was before 5 year (62.8%) and before 1 year (53.6%). Twenty nine of 78 cases of preauricular sinuses were on the left, 25 on the right and 24 bilateral. Sings of infectio were infection were seen in 73.0% of patients with preauricular sinus at operation. Only 31.3% of lesions were infected in patients less than one year of age, but 89.5% between 3-5 year and 100% between 5-8 year. Cartilage was present in five patients with preauricular skin tag. Although re-operation due to wound infection was necessary in four cases, no recurrences were found. The preauricular sinus is a common anomaly in childhood, and has had a relatively high recurrence rate. But most of the recurrence were due to incomplete resection because of combined infection. Initial proper diagnosis and early operation are very important. Identification of the exact anatomical location of sinus tract is necessary because total excision of the lesions including those tracts is the only way to prevent recurrence.
Cartilage ; Classification ; Diagnosis ; Embryology ; Female ; Head ; Humans ; Male ; Neck ; Recurrence ; Skin* ; Wound Infection

Gastric duplication is a rare anomaly which account for only 3.8% of all gastrointestinal duplication. Gastric duplications are usually cysticlesion without communication with lumen. Most frequent presentation is an abdominal mass with vomiting, mainly diagnosed within the first year of life. Surgical removal is necessary in all cases, and optimal timing for surgery is the time that diagnosis is made. However, prenatally diagnosed gastric duplication is getting more common, and determining timing for surgery is not easy due to absent or minimal symptoms just after birth. We experienced prenatally diagnosed gastric duplication in a female newborn baby that gastric duplication was suggested in 24th week of gestational age through prenatal ultrasonogram. Surgical removal was done at 3 months after birth, and showed good results. We think that natural history of gastric duplication and prevalent age of surgical disease which is similar to gastric duplication such infantile hypertrophic pyloric stenosis should be considered when timing of surgery on prenatally gastric duplication is decided.
Female ; Gestational Age ; Humans ; Infant, Newborn ; Natural History ; Parturition ; Pyloric Stenosis, Hypertrophic ; Vomiting