A Case of Dieulafoy's Disease in a Child

Dieulafoy's disease, a vascular anomaly mainly in the upper stomach, is a rare but potentially life-threatening cause of upper gastrointestinal bleeding. Pathogenesis is still controversial, but the most accepted theory is that a persistent caliber vessel in the submucosa is exposed by a small mucosal erosion leading to massive bleeding. The bleeding site is usually within 6 cm of the esophagogastric junction in the cardia or fundus of the stomach. The treatment of choice is therapeutic endoscopy or surgery. The age of patients reported is mainly between 50 and 70 years, and patients of pediatric age are extremely rare. We are reporting a 5-year-old male patient who had Dieulafoy's disease which was diagnosed by emergency upper gastrointestinal endoscopy. Endoscopic finding was a nodular lesion with an adherent clot on the lessor curvature of the stomach 2 cm below the esophagogastric junction. Epinephrine and Beriplast^® was injected in the lesion. On the second day after endoscopic sclerotherapy, the patient had recurred massive hematemesis and accompanying shock. So we performed gastrotomy and ligation. After the operation, he showed an improved general condition and was discharged at the 12th hospital day.ope

Clinical Study of Multiple Intestinal Ulcerations and Perforations Caused by Methicillin-Resistant Staphylococcus aureus in Infants

Purpose: In recent years, the authors experienced a distinctive clinical entity of multiple intestinal ulcerations and perforations in infants. The purpose of this study was to describe the clinical characteristics of this entity, examine th possible pathogenesis and the effective treatment. Methods: Seven infants underwent abdominal exploration under suspicion of surgical abdomen and were noted to have multiple intestinal ulcerations and perforations without evidence of necrotizing enterocolitis by the members of the Korean Association of Pediatric Surgeons. The clinical courses, operative findings, bacterial cultures, pathologic findings, treatment and prognosis of these seven cases were evaluated retrospectively. Results: The characteristics of this entity are as follows : The initial presenting symptoms were all different. Despite conservative treatment with a broad spectrum of antibiotics, diarrhea and abdominal distention developed and progressively grew worse. At laparotomy, all patients exhibited numerous typical transverse linear ulcerative lesions with pin-point perforations scattered mainly in the small bowel. Histologic evaluations of the resected specimens revealed mucosal ulcerations with neutrophil infiltration, submucosal microabscesses and colonies of gram-positive cocci. Methicillin-resistant Staphylococcus aureus(MRSA) was the predominant organism cultured from the body fluid. Only two cases, the completely-resected one and the one immediately treated with vancomycin after operation, survived. Conclusion: We think this distinct clinical entity exhibiting multiple intestinal ulcerations and perforations is caused by MRSA occurring in infants. It has a high mortality because it is very difficult to diagnosis in young infants before laparotomy. Vancomycin is highly effective in treating MRSA, so we think the early diagnosis of this entity can make the treatment successful.ope

The Usefulness of Magnetic Resonance Cholangiography in the Diagnosis of Biliary Atresia

Purpose : To evaluate the usefulness of magnetic resonance cholangiography(MRC) for the diagnosis of biliary atresia in infantile cholestatic jaundice. Methods : Fifty consecutive infants with cholestatic jaundice underwent single-shot MRC for 3 years. The radiologic diagnosis of non-biliary atresia with MRC was based on visualization of the common bile duct and common hepatic duct. The diagnosis of biliary atresia was based on non-visualization of either the common bile duct or common hepatic duct. The final diagnosis of biliary atresia or non-biliary atresia was made with operations or clinical follow-up until jaundice resolved. Results : MRC could clearly visualized the gallbladder, cystic duct. common hepatic duct, common bile duct, both intrahepatic ducts and second order intrahepatic ducts in small neonates and infants. MRC had accuracy of 98%, sensitivity of 100% and specificity of 96% for diagnosis of biliary atresia as the cause of infantile cholestatic jaundice. Conclusions : MRC is a very reliable noninvasive imaging study for diagnosis of biliary atresia in infants with cholestatic jaundice.ope

A Case of Segmental Nectotizing Jejunitis

Segmental necrotizing jejunitis is characterized by severe abdominal pain of acute onset, bilious vomitings and foul smelling loose stools containing blood. Pathologic features include circumferential intestinal wall inflammation ranging from edema with minimal congestion to severe congestion, hemorrhage with necrosis, ulceration, and gangrene with perforation. Early diagnosis and suitable supportive measures prevent unnecessary laparatomy and complications. There was no report of this disease entity in children in Korea. We experienced a case of segmental necrotizing jejunitis with fever, abdominal pain and bloody stools, which was diagnosed by exploration and was treated successfully by antibiotics and supportive measures.ope

Monozygotic Origin of Conjoined Twins

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A Case of Cystic Lymphangioma of the Scrotum and Retroperitoneum was Detected in Fetus

Cystic lymphangioma is a relatively rare congenital malformation of the lymphatic system. It is very rare that retroperitoneal cystic lymphangioma was detected by prenatal ultrasonography. There has been no case reported that scrotal cystic lymphangioma was detected by prenatal ultrasonography. We detected a cystic structure with multiseptation in the scrotum at 30 weeks gestation. In addition, we detected the same structure in the retroperitoneum at 35 weeks gestation by routine ultrasonography. Because of increasing size of the cyst, we performed induction delivery at 38 weeks gestation. Operation of the neonate was performed on the 13th day after birth by a pediatric surgeon and the mass was excised and confirmed as cystic lymphangioma. We experienced a case of huge cystic lymphangioma of the retroperitoneum and scrotum by prenatal sonography and report our case with a brief review of literature.ope

A Case of Spontaneous Perforation of the Common Bile Duct in Infant

Spontaneous perforation of the biliary duct is a rare disease that must be treated immediately when diagnosed in infancy. This disease must be suspected in a previously healthy infant who suddenly has abdominal distension, ascites, and intermittent jaundice. The best way to diagnose the leakage of bile in a clinically stable infant is to use radionuclide hepatobiliary scan. In most cases, however, the diagnosis of the perforation of common bile duct is frequently made during the procedure of operative cholangiogram. The prognosis is usually good with early diagnosis. We experienced an infant of 4 months of age who presented with sudden abdominal distension, jaundice and acholic stool, and confirmed the diagnosis of perforation of common bile duct through operative cholangiogram with t-tube insertion. We report a case of spontaneous perforation in an infant with review of the literatures.ope

Rapidly Evoluting Congenital Cystic Neuroblastoma in a Neonate

Perinatal detection of neonatal suprarenal masses has increased. Here, we report an unusual case of an adrenal cystic neuroblastoma that presented as a purely cystic lesion upon initial postnatal ultrasonography (US) and showed rapid evolution to a mixed cystic and solid mass during follow-up US and MRI. We suggest a short-term (two weeks) follow-up US for neonatal adrenal cystic lesions, even if they appear as purely cystic.ope

Postpyretic Gastroparesis

Gastroparesis is a clinical term for gastric dysmotility or paralysis that presents without mechanical obstruction, but with functional obstruction. Nausea, vomiting, abdominal discomfort and abdominal distension may result from the functional obstruction of gastroparesis. Gastroparesis is frequently associated with such systemic diseases as diabetic mellitus and scleroderma or with certain operations such as vagotomy. Yet gastroparesis is rarely described in older children after viral infection. The authors observed a case of gastroparesis after pyretic symptoms. We report here on this case and its clinical consequences.ope

The Impacts of the Prenatal Ultrasonogram on the Pediatric Surgery

Purpose: Prenatal diagnosis of congenital anomalies provides the information for the perinatal treatment, which can be beneficial to the patients. Yonsei University is one of the largest tertiary referral centers in Korea and its achievement in pediatric surgery is representative figure of pediatric surgery in Korea. This achievement is used to assess the impacts of prenatal ultrasonograms on the doutcomes of prenally diagnosed anomalies in the neonates. Methods: Between 1991 and 2000, 41,458 prenatal ultrasonograms were performed on the pregnant women and the fetal abdominal abnormalities were suspected in 165 fetuses. Of these, 87 fetuses were delivered and the abnormalities were finally confirmed. Theses 87 fetuses was the basis of this study in terms of their prenatal and final diagnosis with the outcomes. Results: Among 87 fetuses, 17 cases were terminated in relation to the maternal health or multiple anomalies. Of the remaining 70 fetuses, 55 patients survived. Among the prenatal diagnoses of 87 fetuses, final diagnosis were made from 75 fetuses and the accuracy of the prenatal diagnosis was found to be 60.0% (45/75). Surgical correction was necessary in 44 cases to confirm the diagnosis and of these, 40 patients survived after the surgical correction. Conclusion: Prenatal diagnosis of the congenital anomalies will improve postnatal outcomes by proper surgical management. However, it can affect the rate terminations of pregnancies even though its accuracy is not so high. To advance the knowledge of the fetal pathophysiology, pediatric surgeons must play an important role in the prenatal diagnosis in relation to the postnatal treatment of the anomalies.ope