Purpose
Acute appendicitis is the most common surgical condition of abdomen in children and the diagnosis is still challenging. The aim of this study was to evaluate the diagnostic value of the Alvarado score and computed tomography (CT) scan and to provide guidance for CT scan to reduce the number of CT examinations for clinically suspected acute appendicitis in children.
Methods
We retrospectively analyzed pediatric patients aged below 18 years and screened via abdominal CT for suspected appendicitis at Yeungnam University Hospital between January 1, 2013 and October 31, 2018. The Alvarado scores of all patients were calculated and correlated with pathology and CT findings.
Results
A total of 198 patients aged between 5 and 18 years were reviewed, and 87 patients underwent surgery whereas 81 patients (40.9%, 81/198) were diagnosed with acute appendicitis pathologically. Six patients underwent negative appendectomy (6.9%, 6/87). The mean Alvarado score was 7.3±1.6 in the appendicitis group and 3.9±1.7 in the nonappendicitis group (p<0.01). A total of 79 positive CT cases were detected in the group with clinically suspected appendicitis. The CT scan yielded 8 false-negative and 6 false-positive results. The sensitivity and specificity of CT scan were 90.1% and 94.8%, respectively, and the sensitivity and specificity of Alvarado score were 75.6% and 89.1%, respectively (p<0.01). The proportion of cases with appendicitis carrying an Alvarado score less than 3 was 4.2% (3/71), and that of patients with Alvarado score greater than 8 was 95.8% (46/48).
Conclusion
The Alvarado score facilitates risk stratification of patients and is expected to guide the reasonable use of CT scan especially in patients with low (less than 3) and high (more than 8) scores.

Purpose
Currarino syndrome is a rare disease, and understanding its clinical characteristics is important because it involves complex anomalies and it requires a multidisciplinary approach for treatment. However, the accumulation of treatment experiences is challenging, and studies on this disease remain insufficient. Our study aimed to review the clinical characteristics and treatment of Currarino syndrome at our institution.
Methods
The medical records of patients diagnosed as Currarino triad or Currarino syndrome at the authors institution from 1997 to 2018 were retrospectively reviewed. Thirteen patients were included in this study.
Results
No significant difference in disease prevalence was observed in terms of sex (male:female, 7:6), and the median age at diagnosis was 7 months (1 day to 35 years). Currarino syndrome was diagnosed during the neonatal period in only one patient, whereas its diagnosis was delayed in the other patients. The most common initial symptom or diagnosis was anorectal malformation (ARM) followed by constipation and sacrococcygeal mass. All patients underwent simple abdominal X-ray and magnetic resonance imaging for the diagnosis of this disease. The most common sacral anomaly was partial sacral agenesis (type III, 62%) followed by hemisacrum (type IV, 38%). The most common presacral mass was mature teratoma. No pathologic malignant features were observed. In ARM, nine patients had anal stenosis, and posterior sagittal anorectoplasty was the most common operative method for ARM. Twelve patients were followed up beyond the age of three, and the median follow-up age was 8.1 years (range: 3.1–30.0 years). Among the 12 patients, 4 patients did not show symptoms of functional abnormality, whereas 8 patients showed constipation, fecal incontinence, urinary dysfunction, or gait disturbance.
Conclusion
The diagnosis of Currarino syndrome could be delayed when sacral bony anomaly and anorectal stenosis are not given attention. A careful follow up is essential because poor long-term functional outcomes are common.

Purpose
Patients with esophageal atresia (EA) often have feeding problems due to esophageal dysmotility, gastroesophageal reflux, and dysphagia, which can delay growth in children. The purpose of this study was to investigate whether proper growth is achieved during the early childhood of patients with EA and to predict associated factors.
Methods
Clinical data of patients with EA who underwent corrective surgery between 2014 and 2017 were collected retrospectively. We analyzed a total of 17 patients who were followed up for at least two years after surgery, except for patients with long-gap and type E EA. The median follow-up period was 979.0 months. We analyzed the weight, height, and weight-forheight (WFH) as z-scores. Linear regression analysis was performed to determine the factors affecting WFH at two years of age.
Results
Of the 17 patients, 11 underwent open surgery and six underwent thoracoscopic surgery. The median time to full feeding was approximately 14 (range, 12.0–53.0) days. In patients with anastomotic stenosis, esophageal balloon dilatation was performed 1–6 times. There was no mortality in our study, and the median follow-up period was 979 days. The mean height was 49.0 cm, the mean weight at birth was 2.69 kg, and the z-scores were −0.55 and −1.44, respectively. The WFH z-score decreased from −1.66 at birth to −1.82 one week postoperatively; however, it improved to −0.2 after six months. In multivariate linear regression analysis, only WFH at birth was a significant variable for WFH at two years of age.
Conclusion
Patients with EA tend to have lower weight and WFH at birth, which worsened after surgery; however, six months after surgery, both weight and WFH recovered to the 50th percentile. The factors that influence WFH at two years of age were significantly related to WFH at birth.

Purpose
Intense multidisciplinary team effort is required for the intestinal rehabilitation of pediatric patients afflicted with intestinal failure (IF). These include enteral and parenteral nutrition (PN) support, monitoring of complications related to treatment, and considering further medical or surgical options for intestinal adaptation.
Methods
In the intestinal rehabilitation team (IRT) at our center, we have experienced 25 cases of pediatric IF requiring multidisciplinary intestinal rehabilitation. This study is a retrospective review of the collected medical records.
Results
Of the 25 subjects treated, 18 were boys and 7 were girls. At the time of referral to the IRT, the mean age was 1.6 years. Median follow-up was 42.9 months. The causes of IF were short bowel syndrome in 18 cases and motility-related in 7 cases. There are 24 patients alive at last follow-up: 12 patients have been weaned off PN, whereas 12 are still dependent on PN. Median time to weaning off PN was 4.8 months. There were 2 cases of IF-associated liver disease. Fifteen cases of central line associated blood stream infections occurred in 9 patients (0.82/1,000 PN days).
Conclusion
We report the results of multidisciplinary intestinal rehabilitation of pediatric IF patients in a Korean IRT. Further studies are required to improve survival and enteral tolerance of these patients.

Situs inversus, polysplenia and annular pancreas are unusual anomalies individually, and are extremely rare in combination. A 3 years old boy presented with failure to thrive and symptoms of chronic upper gastrointestinal obstruction. After adequate radiological investigations, he was explored and managed with a duodeno-duodenostomy. and is doing well on a 1 year follow-up. The preoperative radiological assessment is of paramount importance, so as to avoid any intra-operative surprises.

Congenital central hypoventilation syndrome (CCHS) with Hirschsprung disease (HD), also known as Haddad syndrome, is an extremely rare disorder. Recent studies have identified the paired like homeobox 2b (PHOX2B) gene as the major gene involved in the development of CCHS. The syndrome is diagnosed when gene analysis confirms a mutation in the involved gene, but making an early diagnosis is difficult because of the rarity of the disease. In this study, we report the case of a newborn male with recurrent hypoventilation and bowel distension. HD was suspected on barium enema, and loop ileostomy was performed. After surgery, the abdominal symptoms gradually improved, but extubation was not possible owing to recurrent respiratory failure. These clinical manifestations were indicative of Haddad syndrome, and genetic testing confirmed the presence of a PHOX2B mutation. The patient was diagnosed with Haddad syndrome on the 11th day after birth.

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas encompasses a wide spectrum of epithelial changes from benign adenoma to invasive adenocarcinoma. IPMN is a premalignant lesion where approximately one-third of the tumors undergo malignant transformation. This disease is prevalent in the elderly population and is rare in youth. In this case we report a 12-year-old boy with symptoms of pancreatitis with cystic lesions consistent with branch duct type-IPMN. This is the third reported case of IPMN in a pediatric patient. Our patient underwent pylorus-preserving pancreaticoduodenectomy, and through this case report we suggest when worrisome features or high-risk stigmata features are observed even in pediatric patients, surgical resection should be considered due to the potential for malignant transformation in the future.

Ingestion of neodymium magnets can cause serious complications in children. Making the decision of treatment is difficult because of nonspecific clinical presentation. We report 2 unusual cases of multiple gastrointestinal perforations with closed loop caused by ingestion of multiple bead-shaped neodymium magnets, which are different from the case caused by ingestion of typical magnets. In the first case, the patient was a 2-year-old boy with multiple jejunal perforations. In the second case, the patient was a 19-month-old girl with gastric and proximal jejunal perforations. Patients presented mild and nonspecific symptoms. Exploratory laparotomy was performed, which led to an uneventful postoperative course in both cases. Popular magnet toys composed of small bead-shaped neodymium magnets have different characteristics from typical magnets. They are enough small to swallow easily with greater magnetic force than typical magnets. When multiple neodymium magnets are ingested, they can attract with greater strength across bowel loops, causing pressure necrosis, perforation. However, patients can only present mild and nonspecific symptoms at the time of visit hospital because perforation sites were sealed by magnets which attract each other with great force. Therefore, surgeons should have a high index of suspicion for gastrointestinal perforation in any patient who has ingested multiple magnets, particularly composed of neodymium, presenting with persistent nonspecific abdominal pain.