Enema-Induced spastic left colon syndrome: An unintended consequence of chronic enema use.

BACKGROUND/PURPOSE: Enemas have become a common practice for treating fecal incontinence and severe constipation. Several patients receiving enemas complained of severe, colicky, abdominal pain during enema administration and complained that the duration for fluid to pass was progressively increasing. Contrast studies showed a startling picture of severe right colon dilatation and a spastic, narrow, left colon. An investigation was started to seek the origin and possible management of this condition. METHODS: Medical and radiologic records were reviewed retrospectively, with emphasis on the type and ingredients of enemas used, the duration the patients had been receiving enemas, and their original diagnosis. A literature review was done on previous reports of this condition and publications related to long-term use of enemas. RESULTS: This series included 22 patients (average age, 19.6 years; range, 8-54) with fecal incontinence due to anorectal malformations (10 cases), myelomeningocele (5), cloaca (2), severe colonic dysmotility (2), Hirschsprung's disease (2), and sacrococcygeal teratoma (1). The average duration of enema use was 13.7 years (range, 4-45). The composition of the enemas included saline/glycerin (six cases), only saline solution (five), saline/glycerin/soap (four), plain water (three), and one case each of molasses/milk, saline/glycerin/soap/phosphate, saline/phosphate, and only phosphate. The enemas were performed in an antegrade fashion in 21 cases and rectally in 1. All patients had a dilated right colon and a narrow, spastic, left, transverse, and descending colon. Four patients underwent colonoscopy, colonic manometry, and mucosal biopsies, which did not help in explaining the etiology of the problem. In the literature, 43 reports mentioned a "long-term follow-up" for the administration of enemas, but we could not find a description of symptoms, such as in our cases. CONCLUSIONS: An intriguing and, to our knowledge, previously unreported complication of chronic enema use is presented. We call attention to an overly concerning complication and report our findings in the hope that they will aid and stimulate more investigations into this condition. Several hypotheses to explain the cause are presented, as well as potential treatment options.

Error traps and culture of safety in anorectal malformations.

INTRODUCTION: Attempting to decrease iatrogenic injuries and preventable harm, safety initiatives have become a priority in surgery. For adult hepatobiliary surgery, it has become common to study and consider "error traps" or common pitfalls that exist for laparoscopic cholecystectomy.1-4 Extending this work to children, we have attempted to apply some of these initiatives by identifying error traps common to the care of patients born with anorectal malformations (ARM). METHODS: Five error traps were identified based on a retrospective analysis of operative records and radiographic studies from 398 re operative ARM cases performed by the authors. Once identified, the authors constructed a specific safety plan for each trap to promote a culture that will hopefully prevent ARM iatrogenic injuries. RESULTS: The identified error traps are: 1) creation of a colostomy too distal in the sigmoid colon, 2) inaccurate distal colostogram and definition of the patient's preoperative anatomy 3) absence of a Foley catheter during the repair of an ARM in males and the hazards of separating the anterior rectal wall from the genito-urinary (GU) tract 4) mismanagement of a post-operative anal stricture following an ARM reconstructive procedure 5) limited or unstructured follow up of these patients. For each of the five traps the authors present suggestions for their avoidance. CONCLUSION: The repair on an anorectal malformation is an elective procedure and while not completely avoidable, there should be little tolerance for iatrogenic injury and preventable harm. A culture of safety should be followed, beginning with a recognition of the common error traps associated with ARM procedures.

Controversies in anorectal malformations.

Anorectal malformations are uncommon but complex congenital anomalies that require an individualised strategy of care for each step of the treatment process. These steps, which include preoperative evaluation, operative reconstruction, and postoperative care, are each unique and vitally important to overall patient outcomes. In this Review, we discuss some of the pivotal decisions that should be made at each stage of patient care. Through this process, we highlight some of the more controversial aspects of caring for patients with anorectal malformation and offer insights into various management philosophies.

Characterization of acute appendicitis in diabetic children.

PURPOSE: Children with diabetes mellitus (DM) who develop acute appendicitis can present a diagnostic and clinical challenge. No studies have examined this population since the advent of multiple dose insulin therapy, computed tomography, and laparoscopic surgery. We sought to characterize these children to identify their differences and how to best care for them. METHODS: We retrospectively examined the medical records of children with a preexisting diagnosis of DM treated for acute appendicitis. Values are presented as the mean±the standard error of the mean, and Student's t-test was used for statistical comparison. RESULTS: We identified 18 diabetic children treated for acute appendicitis, making this the largest series of its kind. These children had similar presentations compared to non-diabetics, with the exception of often presenting without fever (83.3% with an initial temperature <38 C). All children developed significant postoperative hyperglycemia (average high 382±18mg/dL), and most had glycemic control for ≤50% of the hospitalization (14/18, 77.8%), but they otherwise had typical postoperative courses. CONCLUSIONS: Diabetic children with appendicitis are often afebrile at presentation and have serum glucose levels that are difficult to control. Collaboration with pediatric endocrinologists is needed to appropriately manage these children during their hospitalization.

Late onset diaphragmatic hernia complicated by intestinal strangulation.

We present a case of a 7-week-old infant who presented with nonspecific respiratory symptoms that quickly progressed to sudden cardiac arrest as a consequence of late-onset diaphragmatic hernia and intestinal strangulation. Unless discovered as an incidental finding, late-onset diaphragmatic hernia should be considered a surgical emergency.

Management of button battery-induced hemorrhage in children.

OBJECTIVES: Button battery ingestions are potentially life threatening for children. Catastrophic and fatal injuries can occur when the battery becomes lodged in the esophagus, where battery-induced injury can extend beyond the esophagus to the trachea or aorta. Increased production of larger, more powerful button batteries has coincided with more frequent reporting of fatal hemorrhage secondary to esophageal battery impaction, but no recommendations exist for the management of button battery-induced hemorrhage in children. MATERIALS AND METHODS: We reviewed all of the reported pediatric fatalities due to button battery-associated hemorrhage. Our institution engaged subspecialists from a wide range of disciplines to develop an institutional plan for the management of complicated button battery ingestions. RESULTS: Ten fatal cases of button battery-associated hemorrhage were identified. Seven of the 10 cases have occurred since 2004. Seventy percent of cases presented with a sentinel bleeding event. Fatal hemorrhage can occur up to 18 days after endoscopic removal of the battery. Guidelines for the management of button battery-associated hemorrhage were developed. CONCLUSIONS: Pediatric care facilities must be prepared to act quickly and concertedly in the case of button battery-associated esophageal hemorrhage, which is most likely to present as a "sentinel bleed" in a toddler.

An unusual colonic duplication associated with a foreign body and anemia: a tale of 2 colons.

Alimentary tract "duplications" are infrequent and usually present in infancy and childhood. The diagnostic difficulties associated with these congenital anomalies underscore the need for a high level of awareness given the variable spectrum of clinical presentation. We report a child with a colonic duplication who presented with an intestinal foreign body and iron deficiency anemia.

Superior mesenteric artery syndrome in a 16-year-old with bilious emesis.

Superior mesenteric artery (SMA) syndrome describes vascular compression of the third portion of the duodenum and presents with nausea, postprandial vomiting, and epigastric abdominal pain. The syndrome is rare and may be missed if appropriate radiologic studies are not performed or the clinical presentation is atypical. The clinical contexts in which SMA syndrome develops usually involve rapid weight loss, alterations in spine anatomy, or external increases in abdominal pressure. Diagnostic methods for identifying duodenal obstruction by the SMA include upper gastrointestinal barium contrast studies, computed tomography scans, or angiography of the aorta with either contrast or magnetic resonance angiography. Medical therapy relies upon nutritional rehabilitation with either jejunal tube feedings or parenteral nutrition until weight gain results in relief of the obstruction. In instances where this approach fails, surgical correction is necessary, most often with laparoscopic duodenojejunostomy.

S100A8/A9: a potential new diagnostic aid for acute appendicitis.

OBJECTIVES: Diagnosing acute appendicitis is a daunting clinical challenge, as there is no single test that reliably distinguishes acute appendicitis from other etiologies of acute abdominal pain. In this study, the authors examined whether circulating levels of S100A8/A9 could be useful as a marker to aid in the diagnosis of acute appendicitis. METHODS: Plasma samples from emergency department (ED) patients with acute abdominal pain (n=181) were tested using an immunoassay for S100A8/A9. RESULTS: The sensitivity and specificity for S100A8/A9 in diagnosing acute appendicitis were estimated to be 93% (95% confidence interval [CI]=81% to 97%) and 54% (95% CI=45% to 62%), respectively. Negative predictive value (NPV) was 96% (95% CI=89% to 99%), and positive predictive value (PPV) was 37% (95% CI=28% to 47%). Performance characteristics of elevated white blood cell (WBC) count were also estimated: sensitivity 63% (95% CI=47% to 76%), specificity 67% (95% CI=59% to 75%), NPV 86% (95% CI=78% to 91%), and PPV 36% (95% CI=26% to 47%). CONCLUSIONS: This is the first report exploring the relationship between circulating S100A8/A9 and acute appendicitis and establishes proof of concept for this biomarker as a diagnostic test for acute appendicitis. Further studies are indicated to optimize the use of this biomarker, in conjunction with other established approaches.

Primary pancreatic neuroblastoma: an unusual tumor in infancy.

Fetal imaging has dramatically impacted neonatal care by providing an advanced warning of many different congenital anomalies. The advancements and widespread use of fetal imaging has, however, increased the identification of various incidental findings that is creating new challenges for neonatal diagnosis and treatment. We report such a case where a fetal pancreatic neuroblastoma (NB) was incidentally detected by computed tomographic scan of the maternal abdomen. Primary pancreatic NB is a very uncommon childhood neoplasm that, to our knowledge, has never been previously reported in the English language presenting in either the prenatal or neonatal periods. A 21-year-old woman complaining of acute abdominal pain and carrying a 35 weeks' gestation fetus was referred for computed tomographic scan because of the concern of maternal appendicitis. That scan was inconclusive for appendicitis but did incidentally detect a fetal mass in the general region of the pancreas. At 36 weeks' gestation, the fetus developed signs of distress, which prompted a cesarean delivery. Neonatal workup confirmed the presence of an abdominal mass in the region of the pancreas, but precise anatomic localization was not possible. Also noted on neonatal workup were elevated urinary catecholamines consistent with a hormonal active tumor. These findings prompted an abdominal exploration of this neonate, which revealed a solid tumor contained in the distal pancreas. The mass was managed by an uncomplicated distal pancreatectomy. The neonate fully recovered, and histologic diagnosis revealed NB, whereas the postoperative urine catecholamines normalized. This case underscores the unintended clinical challenges created by widespread fetal imaging, while presenting the first prenatally diagnosed case in the English language medical literature and earliest treated patient with pancreatic NB.

Thoracoscopic surgery in childhood cancer.

PURPOSE: Recent advances in minimally invasive surgery, especially thoracoscopy, have allowed many new applications in children. The authors' purpose was to review their experience with thoracoscopic surgery in childhood cancer. They hypothesized that thoracoscopy can be efficacious, safe, and cost-effective and has the potential to change the way we care for children with cancer. PATIENTS AND METHODS: The authors reviewed their thoracoscopic experience of the past 7 years. Thoracoscopic procedures performed included biopsy and resection of masses, resection of lung nodules, biopsy of infiltrates, and lobectomy. Some resections required conversion to open thoracotomy. RESULTS: Sixty-three thoracoscopic procedures were performed on 52 children; 8 required conversion to open thoracotomy and 55 were completed by thoracoscopy alone. The overall success rate was 98.4%. There were three complications and no deaths. The mean surgery time was 1.2 hours, mean length of hospital stay was 1.9 days, and mean number of chest tube days was 0.7. CONCLUSIONS: Thoracoscopic surgery in the treatment of children with cancer can be efficacious, safe, and cost-effective. Mediastinal masses can usually be biopsied and resected by thoracoscopy alone. Conversion to open thoracotomy for a more complete resection can be safely accomplished if needed. Thoracoscopic removal of lung nodules allows more accurate staging and early initiation of chemotherapy. Thoracoscopic biopsy of lung infiltrates can be safely performed in intubated, critically ill children and changed the treatment in all of these patients. Surgery time and days in hospital were decreased compared with historical thoracotomy data.