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A 10-year-old girl was admitted for abdominal pain for 1 week. Morning vomiting with 5 Ascaris and diarrhea with Ascaris were found. Radiograph of the abdomen disclosed no significant abnormality. Abdominal sonogram revealed a normal biliary tree; but mildly enlarged pancreatic thickness, and thickened gallbladder wall. Within the thickened gallbladder wall a linear echogenic structure with worm-like movement suspected of being Ascaris was found. We report this case because pediatric pancreatitis and a gallbladder wall thickened with worm-movement have rarely been reported. Urgent treatment and surgery are required for the very ill child with a tensely distended abdomen or signs of peritoneal irritation. Early diagnosis is very important to prevent further complications. We emphasize the role of sonography in the diagnosis of this case and the prevention of progressive deterioration.

Acute appendicitis is the most common surgically amenable cause of acute abdominal pain in children. We analyzed our past experience of appendectomies in children and present the clinical characteristics of appendicitis in children younger than 3 years of age. A better understanding of appendicitis in early childhood would allow us to achieve an earlier diagnosis.A group of 475 children from 4 months to 15 years of age who underwent appendectomy for appendicitis was studied over a 5-year period from July 1994 to June 1999. Excluding cases with negative pathological findings (n = 34), they were divided into 2 age groups: group I (or = 3 years old) and group II (3 years old). Medical records were reviewed and comparisons between clinical findings, laboratory data, pathology findings, and complications were made.Of 441 cases enrolled in our study, 24 (5.4%) were 3 years of age or younger. Of all children older than 3 years of age, 32 (7.1%) had negative pathological findings compared to children younger than 3 years of age (7.7%). The duration of symptoms prior to diagnosis in group I was 3.6 days compared to group II at 2.0 days. Children from group I frequently showed a higher incidence of fever (90% vs. 53.4%), abdominal distention (50% vs. 9.8%), perforation (50% vs. 40.1%), and missed first impression (29% vs. 2.4%) than those from group II. Children of group I also had a higher complication rate (41.7% vs. 11.5%).In early childhood the symptoms and signs of appendicitis usually are nonspecific. There is a longer duration before diagnosis, more instances of fever and abdominal distention, less right lower quadrant pain, less local tenderness and rebounding pain, and no obturator sign. Close observation and on-going evaluation of patients are essential.

We reviewed the histories of four children (2 boys and 2 girls, aged 6 mo to 10 yr) with surgically proven bezoars, treated from 1991 to 1998. Two had gastric bezoars and two had intestinal bezoars. All four patients presented with gastrointestinal symptoms and none had undergone previous surgery. Three had a history of abnormal ingestion, including one who ate a pickled fruit from the Boraginaceae (locally known as Po-pu-tsu) plant for 1 year. Plain radiographs revealed intestinal obstruction in all four patients. Abdominal sonography disclosed a hyperechoic band-like lesion and acoustic shadow in both cases of gastric bezoar, and these bezoars were subsequently confirmed by gastric endoscopy. Lower gastrointestinal studies showed obstruction considered most likely to be due to bezoar in one case, while computed tomography (CT) revealed a characteristic small bowel mottled gas pattern in another. Surgical treatment relieved obstruction in all four patients. Bezoar should be suspected in patients with intestinal obstruction who have a history of ingestion of unusual substances. The findings of this report suggest that sonography and endoscopy are useful in the diagnosis of gastric bezoar, while CT is useful in the detection of intestinal bezoar.

We identify and discuss risk factors related to prolonged hospitalization, as compared with the average hospital stay (5.1 days) of normal delivery neonates throughout the last 10 years, associated with babies delivered unexpectedly in southern Taiwan.This is a retrospective case series study; those babies delivered unexpectedly, including both pre-term and term infants, were pooled out from normal-birth babies, and their laboratory data and clinical histories were reviewed and discussed.There was a total of 18 babies delivered unexpectedly during the past 10 years. Out of these 18 babies, there was only 1 mortality. The mean gestational age was 38 weeks; the mean birth body weight was 3097 g, and the average hospital stay was 6.2 days. Possible clinical factors related to prolonged hospitalization include the following: hypothermia, hypoglycemia, hyperkalemia, polycythemia, and a relatively high ratio of positive findings of chest X-ray study.The best way to reduce prolonged hospitalization caused by unexpected delivery is by prevention, or if this is not possible, by increasing the knowledge and effectiveness of handling intrapartum accidents. Hypothermia, hypoglycemia, hyperkalemia, polycythemia, and positive chest radiological results were the most common reasons for prolonged hospital stays in unexpectedly delivered neonates. However, due to the limited cases in our study, a larger group study is still needed in the future for more precise statistical information.

Vascular rings are a diagnostic and therapeutic challenge for pediatricians. Many diagnostic modalities contribute to the detection of these rare congenital vascular anomalies. The role of flexible bronchoscopy is still being debated. We present 3 cases to emphasize the usefulness of pediatric flexible bronchoscopy (PFB) in the early diagnosis and postoperative evaluation of vascular rings. In patient 1, PFB was performed before conventional techniques were available. A right aortic arch with a retroesophageal aortic diverticulum and mirror-image branching was later confirmed. In patients 2 and 3, pulmonary artery slings could not be detected clearly by imaging studies before PFB was performed. PFB was arranged again postoperatively for these 2 patients, because of difficulty weaning patient 2 from ventilator support and persistent respiratory distress in patient 3. In conclusion, we expect that more vascular rings will be diagnosed using PFB. This instrument is also useful in making a decision for surgery, and for detecting associated tracheobronchial anomalies preoperatively, assuring appropriate correction intraoperatively, and monitoring the condition of vascular rings postoperatively.

A case of bilateral cystic neuroblastoma with liver metastases in a newborn is reported. CT showed a 10 cm right suprarenal multicystic mass and numerous hepatic cystic masses with intracystic fluid-fluid levels. Multiple smaller cystic lesions were also present in the left adrenal gland. To our knowledge, the CT findings of neonatal bilateral cystic neuroblastoma with liver metastasis and massive acute intracystic haemorrhage has not been previously documented.

Megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) is a rare congenital disorder characterized by a massively enlarged urinary bladder without mechanical outlet obstruction and microcolon, as well as a hypoperistaltic bowel with normal ganglion cell distribution. We report one such case to discuss the findings of antenatal ultrasound and the radiologic and pathologic features of this condition.

We report a very rare case of systemic sclerosis in a 6-year-old girl. She presented with diffuse scleroderma, Raynaud's phenomenon, pulmonary interstitial fibrosis, pulmonary hypertension, and right ventricular failure. The diagnosis was confirmed by skin manifestations, high resolution computed tomography, cardiac catheterization, and anti-nuclear antibodies. Nifedipine, prednisolone, digoxin, and furosemide were given. There was remission of the right ventricular failure and dyspnea, and the skin showed partial improvement. The patient remained asymptomatic for a year. The symptoms of respiratory and right heart failure developed again after an episode of lower respiratory tract infection and she eventually died. We discuss the clinical manifestations, treatment, and outcome.

Recognition of rejection and other hepatic complications by needle biopsy plays a significant role in the management of liver allograft recipients. In this report, 22 pediatric patients (below 18 years old) were selected from the 37 liver transplants. Seven of the 22 cases have an uneventful posttransplant course. The most common cause of allograft injury in these patients appeared to be acute cellular rejection. It occurred in 7 (31.8%) of the 22 cases and was the primary process in 8 of the 25 episodes of liver dysfunction. Other etiologies, such as opportunistic viral infection (3 cases), biliary obstruction (2 cases), preservation injury (1 case), and vascular obstruction (1 case) were less common. Acute graft rejection causing liver dysfunction was associated with a mixed portal inflammation, destruction of the interlobular bile ducts, and varied degree of venous endotheliitis, followed by centrilobular hepatocyte necrosis. Chronic rejection was not seen in our pediatric cases. Cold ischemic injury causing transient graft dysfunction as seen in one of our patients demonstrated focal, limited areas of hepatocyte necrosis, mild centrilobular hepatocyte ballooning, and cholestasis without evidence of bile duct damage. More severe ischemic injury resulted from vascular complication causing diffuse hepatocyte necrosis was seen in one patient with hepatic vein thrombosis. The histologic patterns observed were not pathognomic; however, liver biopsies were helpful in suggesting the probable cause of liver dysfunction and in predicting subsequent allograft recovery when used in conjunction with clinical information, radiologic, and other laboratory tests.

Gastric perforation is a rare abdominal catastrophe which associated with high mortality in newborn infants. From June 1978 to July 1991, twelve cases of neonatal gastric perforation presented at Chang Gung Memorial Hospital. Male to female ratio was 9:3. The most common presenting signs were abdominal distension (100%), feeding intolerance (92%), respiratory distress (67%) and poor activity (58%). All cases had significant symptoms between two and five days of age. All of the abdominal plain film showed pneumoperitonium. The most common site of perforation was the great curvature of the stomach (83%). Among the 10 pathological reports available, 8 cases had ischemic change and 2 cases had hemorrhage and inflammatory cell infiltration. There was a high mortality rate of 58% in this series. Male, hyponatremia (serum sodium130 meq/l) and metabolic acidosis (pH7.3) were poor prognostic factors. This report suggests that early diagnosis and early management before clinical deterioration of the metabolic status may improve prognosis for neonatal gastric perforation patients.

Hydrometrocolpos is a rare congenital anomaly and serious life threatening condition in the newborn infant due to its long-term compression sequelae and associated congenital anomalies. Prenatal diagnosis of hydrometrocolpos by sonogram allows appropriate management during the prenatal and neonatal period. The combination of hydrometrocolpos and polydactyly is the cardinal hallmark feature of McKusick-Kaufman Syndrome. We present a case of congenital hydrometrocolpos due to vaginal atresia combined with polydactyly of both feet, mild atrial septum defect, bilateral hydronephrosis, fetal ascites and polyhydramnios. Pathogenesis and treatment of hydrometrocolpos and its associated congenital anomalies are discussed.

A case of primary hyperparathyroidism in an 11-year-old boy who presented with bowlegs is reported and the literature is reviewed. From the literature, it appears that the pathologic findings, clinical manifestations and sex distribution of primary hyperparathyroidism is different in adults than in children and young infants. In adults, primary hyperparathyroidism is commonly caused by adenoma and is often found unexpectedly on a biochemical screen of the serum. Elderly women are most at risk. Renal stones or other renal complications are more frequently seen in adults than in children. Neonatal hyperparathyroidism is often genetically transmitted and is fatal unless recognized and treated early. The characteristic pathologic change is chief cell hyperplasia of the parathyroid glands. On the other hand, adenoma is the most common pathologic finding in older children. No case of childhood parathyroid carcinoma was found in our review of the literature. In children, primary hyperparathyroidism is more frequent in boys than in girls and osseous lesions are more common than in adults. Prognosis is good if the disease is recognized early and appropriate surgical treatment is received.

On the basis of clinical and laboratory data of 50 patients with adult onset Still's disease (AOSD), we strictly examined 4 diagnostic criteria adopted by different authors: criteria of Calabro, ARA, Medsger and Liu Gui-xin. Our result shows that Calabro's criteria has the best specificity (100%) and a higher diagnosis index (0.90) among the 4 criteria. Liu's criteria provides the best sensitivity (98%), but a lower specificity (86.9%) with a misdiagnosis rate of 14%. The rate of missed diagnosis with the ARA criteria is 35.7%. According to the present study, we recommend Liu's criteria for preliminary screen diagnosis, while Calabro's criteria for confirmation and differential diagnosis. Of special interest is that serum ferritin (SF) determination showed significantly higher level in 20 patients with AOSD (average 1194.5mg/L) than that in 19 patients with other rheumatic diseases (average 94mg/L, P0.001). AOSD patients with active disease have higher SF levels than patients with inactive disease (average 2742.9mg/L291.25mg/L, P0.001). So this test might be useful in diagnosis in AOSD and assessment of disease activity.

Edwards syndrome is the second most common autosomal trisomy syndrome, resulting from an extra chromosome in chromosome 18. Recently we encountered two cases with the following representative features: intrauterine growth retardation, polyhydramnios, low-set ears, micrognathia and clenched hands. Both cases were female newborns and had an abnormal karyotype 47, XX, 18.

Neonatal adrenal hemorrhage is frequently associated with large fetal size, birth trauma owing to difficult labor or delivery, perinatal asphyxia, fetal hypoxia, septicemia or coagulation defect. A case of giant baby with perinatal asphyxia and birth injury complicated with adrenal hemorrhage is presented. The baby was admitted at five days of life due to profound jaundice. Right adrenal hematoma was found by abdominal sonography. The hematoma resolved gradually under supportive management by three months of age as documented by serial sonography. No complication was found. We conclude that abdominal sonography is the modality of choice for initial diagnosis and follow-up of neonatal adrenal hemorrhage, and conservative management is the mainstay of treatment.

One hundred and two cases of poorly differentiated adenocarcinoma were obtained from 71 surgical and 31 biopsy specimens of the stomach. These tumours produced both intracellular and extracellular mucins, which were demonstrated to be neutral mucins, N-acetyl sialomucins, sulphomucins and O-acetyl sialomucins. The majority of tumours (85.3%) secreted two or more kinds of mucins simultaneously, but only a small proportion (14.7%) produced a single kind of mucin. Ultrastructurally, three types of mucin granules were identified; these differed in shape and density. Tumour cells containing intracellular mucins or mucin granules were designated as mucous cancer cells and further subdivided into small, large and goblet mucous cells; tumour cells having neither mucins nor glandular differentiation were designated as undifferentiated cancer cells. These histochemical distinctions, supported by the electron microscopical observations, may be useful to demonstrate early malignant foci in gastric biopsies.