Jin-Cherng Sheu

Meta-analysis: the clinical features of the duodenal duplication cyst.

BACKGROUND Duplication cyst of the duodenum is rare. This study describes a case of duodenal duplication and evaluates its clinical features through a literature review. METHODS A case of duodenal duplication is reported, and related articles published from 1999 to 2009 on PubMed were reviewed. Clinical manifestations, diagnostic examinations, and methods of management were analyzed. RESULTS Including this report, there had been 38 citations in literature that provide adequate descriptions of 47 cases of duodenal duplication cysts. Nineteen (40.4%) were discovered before 10 years of age, whereas 10 (21.3%) were found in the second decade. The remaining 18 patients (38.3%) were older than 20 years. The case number decreased as age increased. Overall, 80% of cases presented with abdominal pain, and 53% were complicated with pancreatitis. CONCLUSIONS The most common symptom in duodenal duplication cysts is abdominal pain with or without nausea or vomiting. The most common complication is pancreatitis. Differential diagnoses of pancreatitis, hepatitis, cholestasis, or intussusception should include duplication cyst of the duodenum.

Prenatal and neonatal intussusception

Abstract Intussusception found in the 1st month of life is rare and usually discussed as one entity, neonatal intussusception, but in fact, includes the intussusceptions occurring both prenatally and neonatally, of which the clinical presentations and results are different. Four full-term babies with prenatal intussusception presenting as intestinal atresia (IA) and three premature babies with neonatal intussusception mimicking necrotizing enterocolitis (NEC) are presented. Prenatal intussusception, as one of the causes of IA produces prominent signs of intestinal obstruction immediately after birth. Preoperative evaluation usually fails to yield a definitive diagnosis, but surgery is usually performed in time and is successful. In neonatal intussusception, full-term infants usually have a pathological lead point and the colon is almost always involved. A barium enema is thus useful in diagnosis. Premature babies, on the other hand, rarely have a colonic component, and the clinical features are insidious and similar to NEC. This results in diagnostic confusion that may lead to a dangerous delay in appropriate surgical correction. A high level of suspicion about this condition in cases diagnosed presumptively with NEC is important. Serial abdominal sonograms may be helpful in the early diagnosis of neonatal intussusception.

Intermittent hydronephrosis secondary to ureteropelvic junction obstruction: clinical and imaging features.

OBJECTIVE. We sought to assess the clinical and imaging findings in intermittent hydronephrosis secondary to ureteropelvic junction obstruction, with particular emphasis on the characteristic ultrasonographic findings. METHODS. This prospective, longitudinal, observational study included all children who had intermittent ureteropelvic junction obstruction and presented with abdominal pain over 6 years. Renal ultrasound was used as an initial screening tool to detect intermittent hydronephrosis. Renal ultrasonography was repeated every 1 to 2 days to record serial changes from the symptomatic to the asymptomatic stage. Their clinical manifestations and imaging findings were studied. RESULTS. Eighteen patients (14 boys, 4 girls) were studied. Most had sharp pain that began acutely and typically lasted for <2 days. Most of the children (16 of 18) had nausea and vomiting that accompanied the pain. The acute episode generally resolved spontaneously and was followed by a pain-free interval that ranged from days to months. Factors that predisposed to an attack included increased water intake, vigorous exercise, or bladder distention. All patients had clearly demonstrable obstruction of the renal pelvis during an acute attack, a finding that diminished or resolved during the symptom-free intervals. During convalescence, all patients had renal pelvic wall thickening on ultrasonography. This finding appeared on the second or third day after a painful episode subsided, persisted for 6 to 9 days, and then disappeared in the symptom-free stage. Pyeloplasty was performed in 17 patients, none of whom had recurrent pain on follow-up. Extrinsic obstructions were found in 9 patients. CONCLUSIONS. The keys to diagnosis are awareness of the syndrome, a detailed history, and immediate and serial imaging studies during painful crises. A thickened renal pelvic wall during convalescence is an important ultrasonic sign of intermittent hydronephrosis.

A Twenty-year Review of Early and Late-presenting Congenital Bochdalek Diaphragmatic Hernia: Are They Different Clinical Spectra?

BACKGROUND Congenital Bochdalek diaphragmatic hernia (CDH) is easily recognized if it is present shortly after birth. However, cases of delayed CDH presentation are more subtle and therefore less easily diagnosed. METHODS We retrospectively analyzed 85 patients who were under 18 and diagnosed with CDH between June 1987 and May 2007. Those diagnosed before 1 month of age were categorized as having early-presenting CDH and those diagnosed after 1 month were categorized as having late-presenting CDH. RESULTS Of the 85 cases, 68 (80%) were early-presenting and 17 (20%) were late-presenting CDH. Respiratory symptoms were more common in early-presenting CDH, while gastrointestinal complaints were more dominant in the late-presenting group, particularly in patients with left diaphragmatic defects. Late-presenting CDH, which was more likely to be associated with chronic gastrointestinal symptoms, was associated with a lower mortality than acute early-presenting CDH with respiratory symptoms, despite the more prompt diagnosis of the latter. The small bowel was the organ most commonly herniated into the thorax. Congenital heart disease was the most commonly associated malformation, but the presence of associated anomalies did not increase mortality. CONCLUSION Early-presenting CDH shows acute symptoms, is readily diagnosed, and requires prompt intervention to prevent death. In contrast, late-presenting CDH shows more subtle symptoms and is more difficult to diagnose. Although the late-presenting CHD has better prognosis, it still requires surgery intervention to alleviate the symptoms. Clinicians must maintain a high index of suspicion for CDH, regardless of its presentation.

Clinical Spectrum of Alimentary Tract Duplication in Children

UNLABELLED Alimentary tract duplication is a rare congenital malformations. Sixteen patients admitted to our pediatric ward were enrolled in a ten-year study over the most recent decade. In this paper, we trace their history and retrospectively analyze sex, age, clinical presentations, duplication size and location, presence of ectopic tissue, complications and additional associated anomalies and prognosis. The duplications were cystic in all cases except one tubular duplication of the colon. The cases included duplication of the stomach in 3 cases (19%); ileum in 12 cases (63%); appendix in 1 case (5%); colon in 3 cases (16%); and rectum in 1 case (5%). The most frequent symptom was intermittent vomiting, half of which occurred before 1 year of age. Other symptoms included abdominal pain (50%), abdominal distension (30%), palpable mass (20%), peritoneal sign (13%), bloody stool (6%), fever (6%), and constipation (6%). The outcome was good, except in 2 patients, 1 of whom died of a perforation of the sigmoid colon duplication, and another who was complicated with multiple congenital anomalies. CONCLUSION In our series, the average time period from the initial onset of symptoms to the diagnosis was 6.9 days, with only one exception (a 17-year-old girl who had habitual constipation). The constipation in the single exception was not a specific presentation, which may have delayed the diagnosis. Pediatric patients with unexplained gastrointestinal symptoms must be diagnosed for the possibility of alimentary tract duplication, even though it rarely occurs. Ultrasonography is helpful in diagnosing a duplication, as it will identify a three-layered image representing the duplication cyst, common wall, and outer bowel wall. These features have proven useful for establishing the correct diagnosis or ruling out alimentary tract duplication where surgery was indicated anyway.

Neonatal gastric perforation: report of 15 cases and review of the literature.

BACKGROUND Gastric perforation among neonates is a rare but frequently fatal condition of uncertain etiology. The aim of this study was to review the clinical course of neonatal gastric perforation and to evaluate possible prognostic factors. METHODS We retrospectively analyzed the medical records of 15 patients with neonatal gastric perforation over a 19-year period. Another 97 patients described in the medical literature, for whom the gestational ages and birth weights were clearly stated, were also reviewed. RESULTS In our series, there were three girls and 12 boys, nine of whom were full-term infants and six preterm infants. The most common initial manifestations were poor activity, abdominal distension, and respiratory distress. The overall mortality was 47% (7/15). Prematurity was the only statistically significant risk factor; 83% (5/6) of premature infants died compared with 22% (2/9) of term babies (p < 0.05). Combining our series with the patients reported in the literature, there were a total of 50 premature infants and 62 term infants. Gastric perforation occurred on postnatal days 2-7 and presented with nonspecific manifestations. The mortality was significantly higher in premature than in term infants (31/50, 62% vs. 16/62, 26%; p < 0.001). A trend towards higher mortality in infants with lower birth weights was observed (>2500 g, 28%; 1501-2500 g, 52%; 1000-1500 g, 60%; <1000 g, 100%). Infants with birth weights <2500 g had a significantly higher mortality than infants with birth weights >2500 g (32/58, 55% vs. 15/54, 28%; p<0.05). CONCLUSION Neonatal gastric perforation is associated with high mortality, particularly in premature infants. There is also a trend towards higher mortality in lower-birth-weight infants.

Experience with distal circular myotomy for long-gap esophageal atresia

From 1980 to 1994, the authors treated 65 cases of esophageal atresia (with or without tracheoesophageal fistula). Among these patients, 15 (23%) underwent pure proximal circular myotomies and five (8%) underwent both proximal and distal circular myotomies. Of the latter five cases, three were Gross type C and two were Gross type A. The gap ranged from 4.5 cm to 6.5 cm (mean, 5 cm). Surgical complications in these five patients included two anastomotic leaks (40%), one anastomotic stricture (20%), and four gastroesophageal refluxes (80%). Three patients eventually underwent fundoplication as an antireflux procedure. Mucosal outpouching was noted in all cases. The myotomy did not adversely affect the esophageal peristalsis and motility on the esophagogram. Esophageal function was similar to that of other children who had esophageal atresia without myotomy. All patients could eat food typical for their age. This limited experience suggests that distal circular myotomy might be a safe adjuvant procedure to achieve primary anastomosis in long-gap esophageal atresia.

Experience with primary laparoscopy-assisted endorectal pull-through for Hirschsprung's disease

From October 1996 to July 2001, 61 patients received primary laparoscopy-assisted endorectal pull-through (LAERPT) for biopsy-proven Hirschsprung’s disease (HD) at Mackay Memorial Hospital. The patients’ ages at operation ranged from 7 days to 8 years. The patients were followed up for an average of 3.8 years (range 1–5.5 years). Major complications occurred in five (8.1%) patients, of whom four had surgical complications (two instances of anastomotic leakage, one colon perforation, and one delayed formation of colovesical fistula) and one had postoperative Salmonella infection-induced colonic stricture. The five patients required a diverting colostomy and a redo-pull-through procedure. Postoperative enterocolitis developed in 13 (21%) infants. All of them had enterocolitis before the diagnosis of HD was established. The majority of the episodes of postoperative enterocolitis were mild and resolved spontaneously, but four neonates were hospitalized with significant systemic manifestations, and two of them needed a second operation to solve the problem. After primary LAERPT, stooling frequency in young infants declined rapidly from 10.5±3.2 to 4.4±1.6 bowel movements per day in the first 3 months and more slowly thereafter. Most of these infants had regular bowel movements one to two times per day 1 year after operation. Continence evaluation of the 43 patients over 3 years of age was graded as normal in 24 (56%) patients and good in 19 (44%) patients. Of the 13 patients between 1 and 3 years of age, 11 (85%) had regular normal stools, and two (15%) had occasional soiling. From the current study, the authors concluded that primary LAERPT is a safe and effective method of managing HD with excellent continence results. Considering the complications of surgery and postoperative enterocolitis, patients with long segment aganglionosis, severe enterocolitis, or prominently dilated colon are not good candidates for primary LAERPT.

Prenatal diagnosis of omphalocele associated with umbilical cord cyst

Background. To study the cytogenetics, ultrasound findings, biochemical screening, perinatal outcome, and associated abnormalities in cases of omphaloceles associated with umbilical cord cysts.

Hepatic lymphangioma : a case report

Abstract A patient presented with a huge, pedunculated abdominal cystic lymphangioma arising from the quadrate lobe of the liver near the round ligament. Microscopically, dilated hepatic ducts with scant liver tissue could be recognized in the main cyst. A review of the literature reveals no previous report of a lymphangioma arising in this manner or from this area.