Worsening anemia associated with volvulus in a stable neonate with intestinal obstruction.

Intrauterine intestinal obstruction complicated by midgut volvulus is a serious life-threatening diagnosis. Immediate surgical intervention is generally the course of action upon diagnosis to prevent morbidity and mortality. We report a case of intrauterine intestinal obstruction where the neonate then presented with an unusual onset of volvulus within the first 12 hours of life. The patient was born with generalized edema, a distended abdomen, and pallor. Unlike many cases, the patient did not present with typical signs of volvulus. Diagnostic imaging preceding delivery and the stable postnatal clinical course did not offer a justification for immediate laparotomy. Less than 24 hours later, the patient's hemoglobin significantly dropped leading to an emergent laparotomy. Findings included a volvulus of the terminal ileum and large amounts of intraluminal blood. Our case report includes an analysis of clinical observations that should be considered so that patients presenting with similar signs receive earlier surgical intervention.

MeCP2 in the enteric nervous system.

BACKGROUND: Rett syndrome (RTT) is an intellectual deficit and movement disorder that develops during early childhood in girls. Affected children are normal until 6-18 months of age, after which symptoms begin to appear. Most cases of RTT are due to mutations in the MeCP2 gene leading to disruption of neuronal communication in the central nervous system. In addition, RTT patients show peripheral ailments such as gastrointestinal (GI), respiratory, and cardiac dysfunction. The etiology of intestinal dysfunction in RTT is not well-understood. Reports on the presence of MeCP2 in the peripheral nervous system are scant. As such we examined the levels of MeCP2 in human and murine GI tissue and assessed MeCP2 expression at various developmental stages. METHODS: Immunohistochemistry for MeCP2, HuC/D, juvenile beta tubulin, and GFAP was performed on human and murine intestine. Western blots of these same tissues were probed with MeCP2, vAChT, nNOS, and beta-actin antibodies. KEY RESULTS: MeCP2 is expressed throughout the GI tract. MeCP2 is expressed specifically in the enteric nervous system of the GI tract. MeCP2 is expressed in the GI tract throughout development with appearance beginning at or before E11.5 in the murine intestine. CONCLUSIONS & INFERENCES: The proof of MeCP2 expression in enteric neurons suggests that the GI dysmotility in Rett may arise from enteric network dysfunction secondary to MeCP2 mutation.

Antenatal hemorrhage of a cervical lymphatic malformation presenting as a draining neck mass: An unusual presentation.

Lymphatic malformations in the neck can present as large fetal neck masses causing airway obstructions with potential perinatal demise and can pose a therapeutic challenge. We present a rare case of prenatally diagnosed large fetal neck mass with features of lymphatic malformation with intralesional hemorrhage of uncertain origin. Postnatal evaluation showed a complex cystic-solid lesion eroding through the skin with an open wound that made it clinically hard to differentiate from a teratoma. Given that malignancy could not be completely ruled out, surgery was favored. Final pathology showed a complex lymphatic malformation with intralesional hemorrhage, despite having no associated capillary, venous or arterial malformations.

Iatrogenic anterior diaphragmatic hernia in childhood.

Congenital anterior diaphragmatic hernias (ADH) account for 2 - 6 % of diaphragmatic defects, whereas acquired ADH are rare. These hernias are most often the result of blunt or penetrating trauma. This is the first report of iatrogenic ADH in childhood. Two children with asymptomatic, iatrogenic ADH were diagnosed at 6 and 12 months of age, respectively. Both had undergone previous cardiac surgery requiring pericardial drainage. The ADH was seen on an incidental chest X-ray in one patient, and during a laparoscopic-assisted gastrostomy in the other. Both were successfully repaired laparoscopically. Subxiphoid pericardial drains may result in iatrogenic ADH. A high index of suspicion after cardiac surgery is necessary to avoid missing this potentially serious complication. Laparoscopy is a useful tool in both the diagnosis and repair of this condition.

Large paraesophageal hernias in children. Early experience with laparoscopic repair.

BACKGROUND: Large paraesophageal hernias (LPEH) in children are unusual. The aim of this study is to report the management of LPEH and our initial experience with the laparoscopic approach. METHODS: Since September 2005, four children aged 4-17 years underwent laparoscopic repair of LPEH at the Children's Hospital of Eastern Ontario. Three children had previous fundoplications and two of these had a gastrostomy. Closure of the hiatal defect included crural sutures and prosthetic material, either polytetrafluoroethylene-polypropylene mesh or porcine small intestinal submucosal patch. The charts were retrospectively analyzed. RESULTS: The operative time was 300-540 minutes, with one conversion and two mediastinal pleural tears. Postoperative chest X-ray showed neither mediastinal nor intrapleural air. The median hospital stay was 3 days. Contrast X-ray showed no recurrent hernia and an intact fundoplication. CONCLUSION: LPEH in children is usually a complication of previous fundoplication. Laparoscopic repair is technically demanding but feasible. Adequate crural repair using mesh may reduce the incidence of recurrence. In view of the rarity of LPEH in children, a combined multicenter study is needed to evaluate the results of laparoscopic repair.

Beware of stapled side-to-side bowel anastomoses in small children.

Side-to-side, functional end-to-end stapled anastomosis (SS-EESA) is a frequently employed technique to re-establish continuity following bowel resection. We describe, for the first time in children, two cases of an important complication of this form of bowel anastomosis. Patient 1 had resection of a jejunal lymphangioma and formation of an SS-EESA at the age of 3 years. By the age of 7 years he was demonstrating symptoms consistent with malabsorption, which was confirmed by hydrogen breath testing. An upper GI contrast study indicated a segmental dilatation of the distal small bowel. Elective laparotomy revealed partial volvulus of a greatly dilated SS-EESA. Patient 2 had undergone bowel resection as a neonate for ileal atresia, with end-to-end anastomosis. An anastomotic stricture developed at two months of age that was resected with formation of an SS-EESA. Multiple ensuing episodes of partial small bowel obstruction were managed non-operatively until, at 5 years of age, she presented with complete bowel obstruction. At operation, volvulus of a hugely dilated SS-EESA was found. Intraoperative cultures of the succus entericus were consistent with bacterial overgrowth. Both patients were successfully treated with resection of the SS-EESA and primary anastomosis. SS-EESA can be complicated by bacterial overgrowth, massive dilatation and volvulus. In patients with SS-EESA who present with recurrent obstructive symptoms, this complication should be considered.

Fast evaluation of intraoperative biopsies for ganglia in Hirschsprung's disease.

PURPOSE: The most important diagnostic features of Hirschsprung's disease (HD) are the combination of aganglionosis and hypertrophic nerve bundles. Acetylcholinesterase staining is widely used for diagnosis of HD as it identifies hypertrophic nerves in both diagnostic and intraoperative biopsies. The main drawback of this method is in the identification of ganglia. It has been suggested that the combination of this method together with another histochemical marker would be a superior diagnostic tool. Hematoxylin and eosin is still the diagnostic measure of choice for identifying ganglia in many centers, although it presents a persistent diagnostic challenge for pathologists trying to rapidly and accurately interpret the frozen biopsies that guide intraoperative decision making. METHODS: Therefore, we sought to develop a fast, intraoperative immunohistochemical protocol for visualization of ganglia and nerves in HD specimens that can be used in conjunction with these other tools. RESULTS: With the use of acetone fixation and immunofluorescence staining with antibodies to neurofilament 68 and tubulin, ganglia in sections of full thickness and suction biopsies could be visualized in only 10 minutes. This protocol facilitated the identification of ganglia in hematoxylin and eosin-stained adjacent sections and also identified hypertrophic nerve trunks. CONCLUSION: This method should significantly enable the identification of ganglia in suction and full thickness biopsies.

Association between weather conditions and the number of patients at the emergency room in an Argentine hospital.

The aim of this paper is to study the relationships between hospital emergencies and weather conditions by analysing summer and winter cases of patients requiring attention at the emergency room of a hospital in the city of Buenos Aires, Argentina. Hospital data have been sorted into seven different diagnostic groups as follows: (1) respiratory, cardiovascular and chest-pain complaints; (2) digestive, genitourinary and abdominal complaints; (3) neurological and psychopathological disorders; (4) infections; (5) contusion and crushing, bone and muscle complaints; (6) skin and allergies and (7) miscellaneous complaints. In general, there is an increase of 16.7% in winter while, for group 2 and group 6, there are more patients in summer, 54% and 75% respectively. In summer, the total number of patients for group 6 shows a significant positive correlation with temperature and dew-point temperature, and a negative correlation with the sea-level pressure for the same day. In winter, the same relationship exists, however its correlation is not as strong. The lags observed between these three variables: maximum dew-point temperature, maximum temperature, minimum air pressure and the peaks in admissions are 1, 2 and 4 days respectively. In winter, increases in temperature and dew point and decreases in pressure are followed by a peak in admissions for group 2. In winter, there are significantly more cases in group 5 on warm, dry days and on warm, wet days in the summer.