• Journal of the Korean Society of Radiology
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• v.85 no.1
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• pp.124-137
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• 2024

Malrotation and midgut volvulus are surgical emergencies that commonly occur within the first month of life. The classic symptom is acute bilious vomiting, while nonspecific symptoms such as recurrent abdominal pain may be present in older children. Malrotation can be associated with duodenal obstruction caused by an abnormal peritoneal fibrous band or congenital anomalies, such as an annular pancreas or a preduodenal portal vein. Volvulus can lead to bowel ischemia and a life-threatening condition, thus prompt and accurate diagnosis is crucial. Diagnosis can be made through upper gastrointestinal series, ultrasonography, and CT, with ultrasonography being preferred as a screening tool due to its rapid and accurate diagnosis, without radiation exposure, in children. This pictorial essay discusses the key imaging findings and diagnostic approaches for malrotation and midgut volvulus, as well as diagnostic pitfalls based on actual cases.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.5
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• pp.487-492
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• 2019

Waardenburg syndrome (WS) type IV is characterized by pigmentary abnormalities, deafness and Hirschsprung's disease. This syndrome can be triggered by dysregulation of the SOX10 gene, which belongs to the SOX (SRY-related high-mobility group-box) family of genes. We discuss the first known case of a SOX10 frameshift mutation variant defined as c.895delC causing WS type IV without Hirschsprung's disease. This female patient of unrelated Kuwaiti parents, who tested negative for cystic fibrosis and Hirschsprung's disease, was born with meconium ileus and malrotation and had multiple surgical complications likely due to chronic intestinal pseudo-obstruction. These complications included small intestinal necrosis requiring resection, development of a spontaneous fistula between the duodenum and jejunum after being left in discontinuity, and short gut syndrome. This case and previously reported cases demonstrate that SOX10 gene sequencing is a consideration in WS patients without aganglionosis but with intestinal dysfunction.

• Advances in pediatric surgery
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• v.16 no.2
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• pp.126-133
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• 2010

Malrotation is a congenital anomaly that becomes symptomatic more frequently during infancy. The indication for surgical treatment at that age is straightforward. In older children, the diagnosis may be more difficult because of chronic and vague complaint. The aim of this study is to compare the symptoms, rate of volvulus and surgical findings in children younger and older than one year. A retrospective study of 40 patients in a a single medical center diagnosed with malrotation from April 1996 to May 2010 was performed. There were 20 (50 %) boys and 20 (50 %) girls. At the time of operation, 27 (67.5 %) patients were younger and 13 (32.5 %) were older than 1 year. Vomiting was seen in 20 cases (74.1 %) of the younger group compared to 2 cases (15.4 %) of the older group. Abdominal sonography and upper gastrointestinal series showed a sensitivity of 100%. Operative findings: 12 (44.4 %) of the younger group presented with volvulus compared to none of the older group. The Ladd's procedure was routinely performed with appendectomy in all cases and bowel resection was requires when volvulus included bowel necrosis or other anomalies were found. After definite procedures, surgical correction for adhesive obstruction was necessary in 5 menbers (18.5 %) of the younger group and 1 patient (7.7 %) in the older group. There was 1 death due to respiratory failure and pneumonia. Abdominal pain was more frequent symptom and bilious vomiting was less frequent. Volvulus did not occur in the older group. Malrotation should be diagnosed promptly in children over 1 year of age by upper gastrointestinal series and abdominal ultrasonography even though symptoms are not as clear cut as in infants.

• Journal of Yeungnam Medical Science
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• v.4 no.1
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• pp.33-42
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• 1987

Congenital origin intestinal obstruction are important disease due to required emergency operation. So accurate and rapid diagnosis is needed for decreased mortality and morbidity. Radiologist must detect to accurate obstruction site and also associated other congenital anomalies. And also embriological bases are very important role to the diagnosis of theses diseases. We were analysed radiologically and clinically 25 cases with congenital origin intestinal obstruction with review of literature. 1. Hypertrophic pyloric stenosis 6 cases, midgut malrotation 4 cases, congenital megacolon 8 case, imperforated anus 5 cases, ileal atresia 1 case and duodenal atresia 1 case. 2. Male and female radio were 16:9. Especially on hypertrophic pyloric stenosis, 5 cases were male infants. 3. All cases of hypertrophic pyloric stenosis represented string sign and also pyloric beak sign shoulder sign on UGI. 4. I case duodenal atresia showed double bubble sign on simple abdomen x-ray and ileal atresia showed mechanical small bowel obstruction sign with microcolon. 5. On midgut malrotation, cecum was located in right upper abdomen on 4 cases. And 2 cases were associated with Ladd's band. I case with volvulus and 1 case with mesenteric defect. 6. Involved site of all congenital megacolon were localized to rectosigmoid colon. 7. On 5 cases imperforated anus, 3 cases were low type and 2 case high type. Rectoperineal and rectourogenital fistula were demonstrated on 4 cases.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.16 no.2
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• pp.131-134
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• 2013

Cecal volvulus is uncommon in pediatric patients and there are few reports of cecal volvulus with cerebral palsy. Here, we report the case of a 19-year-old male patient who presented with abdominal distension, a history of cerebral palsy, refractory epilepsy due to lissencephaly, and chronic constipation. An abdominal x-ray and computed tomography without contrast enhancement showed fixed dilated bowel intensity in the right lower abdomen. Despite decompression with gastric and rectal tube insertion, symptoms did not improve. The patient underwent an exploratory laparotomy that revealed cecal volvulus. Cecal volvulus usually occurs following intestinal malrotation or previous surgery. In this patient, however, intestinal distension accompanying mental disability and chronic constipation resulted in the development of cecal volvulus. We suggest that cecal and proximal large bowel volvulus should be considered in patients presenting with progressive abdominal distension combined with a history of neuro-developmental delay and constipation.

• Journal of Chest Surgery
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• v.24 no.12
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• pp.1238-1241
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• 1991

Agenesis of the hemidiaphragm is unusual congenital anomaly associated with a high mortality. A case of congenital agenesis of left diaphragm was experienced in 22-day old male patient who was dyspneic and cyanotic on admission. Emergency exploration through the left eight interspace thoracotomy showed complete agenesis of the left diaphragm. The stomach and transverse colon covered with peritoneal sac was partially herniated into left hemithorax. The left lung was slightly hypoplastic. This neonate had no intestinal malrotation. The defect was reconstructed using Dacron graft. Dacron patch was sutured with interrupted Ethibond to chest wall anteriorly, esophagus aorta and costomediastinal sinus medially, and the tenth rib posterolaterally. Postoperatively, Extubation was performed at 1st day, but some respiratory difficulty was noted. Severe dyspnea was occurred at postoperative 11th day and so reintubation was done. Intermittently ventilatory support and intravenous alimentation were continued for 9 days after that. Thereafter he had no respiratory problems at discharge.

• Korean Journal of Radiology
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• v.23 no.1
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• pp.124-138
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• 2022

Gastrointestinal (GI) emergencies in neonates and infants encompass from the beginning to the end of the GI tract. Both congenital and acquired conditions can cause various GI emergencies in neonates and infants. Given the overlapping or nonspecific clinical findings of many different neonatal and infantile GI emergencies and the unique characteristics of this age group, appropriate imaging is key to accurate and timely diagnosis while avoiding unnecessary radiation hazard and medical costs. In this paper, we discuss the radiological findings of essential neonatal and infantile GI emergencies, including esophageal atresia and tracheoesophageal fistula, hypertrophic pyloric stenosis, duodenal atresia, malrotation, midgut volvulus for upper GI emergencies, and jejunoileal atresia, meconium ileus, meconium plug syndrome, meconium peritonitis, Hirschsprung disease, anorectal malformation, necrotizing enterocolitis, and intussusception for lower GI emergencies.

• Advances in pediatric surgery
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• v.10 no.2
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• pp.112-116
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• 2004

Midgut volvulus secondary to intestinal malrotation usually presents within the first month of life. Diagnostic delay may result in midgut infarction and mortality. In this retrospective study, we review seventeen cases of midgut volvulus to assess the importance of early recognition for midgut volvulus in pediatric patients of any age.. These patients were diagnosed as having a midgut volvulus by operation at Ewha Womans University Hospital. Eleven patients (64.7 %) were less than 1 month of age, and fifteen were boys (88.2 %). The mean gestational age was 38.3 weeks and the birth weight was 3.1 kg. Eight patients (47.1 %) had one or more combined anomalies such as heart malformation, brain ischemia, Down's syndrome or duodenal atresia. Vomiting was the most common symptom. Only thirteen patients underwent preoperative diagnostic procedures; 13 abdominal sonography demonstrated the whirlpool sign in 8 patients, upper gastrointestinal tract roentgenography showed a cork-screw pattern in 7 patients, and barium enema or small bowel series demonstrated positive findings in 7 patients. A Ladd's procedure was was formed on all patients.. There was no mortality or severe morbidity such as short bowel syndrome. Midgut volvulus should be included in the differential diagnosis in any infant or child who presents with the symptoms of acute abdomen, especially with vomiting.

• Advances in pediatric surgery
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• v.9 no.2
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• pp.81-88
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• 2003

Congenital dilatation of the common bile duct (choledochal cyst) is an uncommon disease. Although the etiology is unknown yet, various theories such as distal obstruction of the common bile duct, congenital weakness of the duct and anomalous union of the pancreaticobiliary duct have been offered to explain the occurrence of choledochal cyst. Thirty - six cases of choledochal cyst over 22 years were analyzed clinically and classified according to Todani's classification and Kimura's anomalous union of pancreaticobiliary duct type. Todani type 1 consisted with 22 cases which were subdivided into 19 cases of type Ia, 1 case of type Ib and 2 of type Ic. Type IVa consisted with 14 cases including one case of Caroli's disease. There were 25 type BP cases and 10 type PB cases and 1 normal pancreatobiliary junction. Serum alkaline phosphatase was increased significantly in almost all cases. Seven patients (19.4%) had associated congenital anomalies such as double gallbladder, left - sided gallbladder, common bile duct web, biliary atresia, accessory hepatic duct, heterotopic pancreas, cleft lip and 2 cases of intestinal malrotation. All patients underwent cyst excision and Roux - en - Y hepaticojejunostomy and cholecystectomy. There was one death due to methicillin resistant Staphylococcus aureus sepsis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.6
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• pp.608-612
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• 2019

Gastric volvulus (GV) is an uncommon pathology, with 10-20% of cases occurring in children, typically before one year of age. It often occurs in people with congenital diaphragmatic hernias, intestinal malrotation, eventration of the diaphragm, paraesophageal hernias, wandering spleens, asplenism, or intra-abdominal adhesions. We report a rare case of chronic GV after left hemihepatectomy for hepatoblastoma in a child. The patient was a 9-year-old boy who complained of upper abdominal pain and postprandial upper abdominal distension for one year. At the age of 4 months, he was diagnosed with hepatoblastoma and had undergone left hemihepatectomy. The upper gastrointestinal contrast study revealed chronic organoaxial gastric volvulus. After a surgical procedure involving adhesiolysis and an anterior wall gastropexy, the patient improved and the symptoms resolved. Although GV is a rare disease, it should be suspected in a patient with a previous abdominal surgical history who is complaining of abdominal distension and pain.