BACKGROUND:Familial dysautonomia (FD) is a hereditary disease of the autonomic and sensory nervous system. A prominent manifestation of FD is gastrointestinal dyscoordination, which contributes to the morbidity and mortality in FD.AIM:As the myenteric plexus is an essential factor in gastrointestinal motility control, we compared its morphology in appendices of FD patients and controls.METHODS:Appendices from FD patients (N=19) were obtained during surgery of fundoplication and gastrostomy; normal appendices (N=17) were obtained from patients suspected to suffer from acute appendicitis, in whom, however, the appendix was found to be normal. Specimens were stained histochemically for NADPH diaphorase (NADPH-d) and in a blinded manner examined under a light microscope for seven morphologic parameters: ganglionic density, neuronal density, ganglionic area, number of stained neurons per ganglion, nerve bundle width, ratio between nervous tissue area and total area, and neuronal area.RESULTS:Ganglionic density was 10.13 per mm(2) in controls versus 5.01 per mm(2) in FD (p<0.05). Neuronal density was 70.12 per mm(2) in controls, compared with 22.09 per mm(2) in FD (p<0.01). The other parameters were not different between the two groups.CONCLUSION:Densities of myenteric ganglia and neurons of FD patients were significantly lower than in controls. This deficiency may contribute to the pathogenesis of FD gastroenteropathy.

Aging is believed to affect the structure and function of the enteric nervous system, but little specific information on this topic is available, particularly in humans. The aim of this study was to investigate the effect of age on the structure of myenteric ganglia in the human colon. We examined myenteric ganglia in colonic specimens obtained from 168 patients aged 10 days to 91 years. Nerves were stained in whole mount preparations using the vital fluorescent dye 4-(4-dimethylaminostyryl)-methylpyridinium iodide (4-Di-2-ASP) and other staining methods. Human myenteric ganglia were classified into three types: normal, those containing empty spaces (‘cavities’) and those containing large nerve fiber bundles. We found a statistically significant increase with age in the proportion of ganglia with cavities. Conversely, there was a decrease with age in the proportion of normal ganglia. The proportion of fiber-containing ganglia did not change with age. These findings indicate that there is an increase with age in the number of abnormally appearing myenteric ganglia in the human colon, which may contribute to the disturbed colonic motility in the aging population.

OPIOIDS are known to reduce the motility of both the colon and the small intestine. However, some investigators have reported, both in animal models and in humans, that morphine increases duodenal motility. In vivo studies of the effect of morphine on small bowel motility are complicated because most have been performed in the postoperative period, where the additional trauma of laparotomy and bowel manipulation had already affected bowel motility. Previous studies have reported that intestinal ischemia also causes prolonged inhibition of bowel motility. The effect of morphine on intestinal motility has not been well assessed in situations in which ischemic injury to the bowel occurs without concurrent surgical injury. Recently, Zhang et al. reported that in a rat model of intestinal ischemia and reperfusion, pretreatment with morphine before ischemia and reperfusion markedly attenuated intestinal injury. The aim of the current study was to evaluate the effect of morphine on small bowel propulsion activity in a rat model in which controlled bowel ischemia was caused without concurrent abdominal surgery.

Still debated are the appropriate techniques for the repair of abdominal wall defects and the methods used to measure their strength. Although tension has been used in many studies to test wound strength, bursting pressure reflects more accurately the pathophysiology of wound dehiscence. The aim of the current study was to evaluate three different techniques for closure of abdominal wall defects using a new and more accurate device for bursting pressure measurements. Full thickness abdominal wall defects measuring 2 cm(2) were created in 43 anesthetized rats randomly assigned to three groups: simple primary closure (n = 15), Mayo repair (n = 14), and primary closure reinforced with a mesh (n = 14). Thirty days after surgery, the rats were sacrificed. The abdominal wall was fully excised and placed over a bursting chamber made of a metal cylinder connected to a carbon dioxide source with a control valve and a manometer. Gas was gradually released while the pressure was recorded until bursting occurred. Disruption of all closures occurred at the point where the suture itself penetrated the tissue. The average bursting pressure was 1383 +/- 299 mm Hg for the primary closure group, 1200 +/- 409 mm Hg for the mesh reinforcement group, and 1607 +/- 337 mm Hg for the imbrication repair (Mayo) group (P < 0.03). The data suggests an advantage for the Mayo repair over the other two repairs. The bursting chamber tested is a new and more reliable method to study techniques and conditions influencing the strength of abdominal wall closure.

A solid mass in the umbilical cord is a very rare finding. A neonate with isolated ectopic liver tissue found in the umbilical cord is reported. This is the first known such reported case.

The aim of this study was to determine the contribution of intestinal motility and cecal bacterial overgrowth to liquid diet-induced bacterial translocation (BT). Three different commercially available liquid diets were offered to mice for 1 week. BT to the mesenteric lymph nodes (MLN), spleen, and liver were examined as well as cecal bacterial counts and populations, small bowel length and weight, and histopathologic changes in the ileal and jejunal mucosa. In addition, the effect of the various diets on intestinal motility was measured by the transit index of a charcoal mixture introduced into the stomach. The incidence of BT to the mesenteric lymph nodes was significantly and similarly increased (p < .05) in mice fed Vivonex (30%), Ensure (30%), and Osmolite (33%) compared with chow-fed controls (0%). Compared with chow-fed controls, all three liquid diets were associated with the development of cecal bacterial overgrowth (p < .01). There were no significant changes in the transit index for the three liquid diet groups compared with the chow-fed controls. BT to the MLN was induced by all three liquid diets tested, casting some doubts as to their role in preventing BT in clinical use. BT was associated with a statistically significant increase in cecal bacterial count but was not associated with gut motility changes in this model. In fact, no significant changes in intestinal motility were noted in all groups tested.

Following the great success and wide acceptance of laparoscopic surgery, the mini-invasive approach has been adopted for use in thoracic surgery. Thoracoscopic surgery is gaining acceptability as the procedure of choice for the treatment of recurrent pneumothorax and bullous lung disease, peripheral benign and malignant lesions, diffuse pulmonary infiltrates, mediastinal and pleural lesions, esophageal surgery, and major pulmonary resections for primary lung tumors. We present the 4-year experience of a general surgery service that extended the use of its advanced laparoscopic skills to the performance of thoracoscopic surgery in 80 patients. Using thoracoscopy, we performed biopsy or excision of pulmonary lesions (23 patients), spontaneous pneumothorax (14 patients), thoracic sympathectomy (41 patients), and Heller's esophagomyotomy and pericardiotomy (1 patient each). The results are excellent, and we believe the procedures presented in this series, and similar ones, can be accomplished safely and successfully by well-trained laparoscopic surgeons.

Primary pulmonary fibrosarcoma is a rare malignant tumor in childhood. In the absence of metastases, complete resection is curative. An 8-year-old boy suffered from unresolving pneumonia due to an obstructing lesion in the left main bronchus. Cytology of the bronchoalveolar lavage fluid and histology of bronchial biopsy revealed the diagnosis of pulmonary fibrosarcoma. The tumor did not respond to chemotherapy, and a total lobectomy with sleeve resection was performed with complete removal of the neoplasm. Two years after the operation the child has no evidence of disease.

until PO intake was 2.1 days for the group without NG decompression and 3.2 days for the group with.Length of hospital stay was 4.6 days for the group with and 4.5 days for the group withour.There was no significant difference in the rate of wound infection or development of ileus.None of the patients developed an anastomotic leak.More importantly, of the 13 patients with NG tubes, 7 (54%) had one or more documented episodes of desaturation (<89%) post-op, compared to only 9 (26%) in the group without NG tube.Of the 7 patients in the NG tube group, only 1 (14%) had documented sleep apnea, whereas, 5 (56%) did so in the no-NG group. Conclusion:The routine omission of nasogastric decompression in morbidly obese patients following gastric bypass surgery can be safely performed.Furthermore, this select group of patients may derive greater benefit since the potential morbidity associated with routine nasogastric decompression is likely greater.

Background —Free radicals are associated with post-ischaemic intestinal injury and contribute to major clinical problems primarily in premature infants. Various antioxidative means and modes of intervention, previously tested, have demonstrated only limited efficacy. Aims —To study the protective activity of the stable nitroxide radical 4-OH, 2,2,6,6-tetramethylpiperidine-1-oxyl (TPL) and its respective hydroxylamine (TPL-H) against ischaemia/reperfusion (I/R) injury. Methods —An isolated loop of ileum was created in laboratory male Sabra rats and constantly perfused with warmed normal saline. Intestinal injury was elicited through clamping of the superior mesenteric rat artery followed by reperfusion. Either TPL or TPL-H was given intravenously immediately before ischaemia or reperfusion and continuously afterwards. The rate of mucosal to lumen clearance of para-aminohippurate (PAH) was used to evaluate intestinal mucosal injury. Serum and perfusate levels of both TPL and TPL-H were measured using electron paramagnetic resonance spectrometry. Results —The increase in intestinal permeability induced by I/R was significantly inhibited by both TPL and TPL-H. The nitroxide was effective also when given immediately before reperfusion. Conclusion —Through a continuous exchange, TPL and TPL-H act as self-replenishing antioxidants and thus protect from intestinal injury. This demonstrates the potential of the family of nitroxide antioxidants against oxidative stress in general and I/R injury in particular.

Intestinal mucosal injury of various degrees occurs in many clinical situations and is initially evidenced by altered mucosal permeability. The latter may be assessed in animal models by determination of plasma-to-intestinal lumen clearance of specific molecules, usually chromated 51Cr EDTA. The purpose of this study was to evaluate the usage of para-aminohippurate (PAH) as a substitute for the commonly used radioactive material, i.e., 51Cr-EDTA, in the evaluation of intestinal mucosal injury. An isolated loop of ileum was created in rats and constantly perfused with warmed normal saline. Both renal pedicles were ligated. Either 51Cr-EDTA (18.5 Bq/kg) or PAH (58 mg/kg) was injected IV. Fifteen-minute intestinal ischemia was produced by clamping the superior mesenteric artery immediately after the end of an equilibration period. The perfusate was collected for 10 min prior to the initiation of intestinal ischemia, during the last 10 min of ischemia, and during the following three 10-min periods of reperfusion. Blood samples were collected at the end of each collection period for the determination of either PAH or 51Cr-EDTA concentrations and the calculation of either PAH or 51Cr-EDTA plasma-to-lumen clearances. PAH and 51Cr-EDTA plasma-to-lumen clearances followed the same pattern in all five assessed periods with no statistical difference between the two. PAH plasma-to-lumen clearance is a feasible, reliable, and inexpensive method for the evaluation of ischemia/reperfusion injury to the intestinal mucosa. It can safely replace the commonly used method in animal models that utilizes radioactive materials such as 51Cr-EDTA.

BACKGROUND:Antireflux operations have been recommended for infants and children suffering from complications related to gastroesophageal reflux (GER). In recent years, the laparoscopic approach has been used increasingly for antireflux surgery in adult patients. This is our initial experience with Nissen fundoplication in infants and children under 2 years of age.PATIENTS:We operated on 11 patients weighing between 3.0 and 10.0 kg. The main indications for surgery were GER-induced aspiration pneumonia and failure to thrive, in spite of intensive conservative treatment. All patients except one had an associated neurological abnormality, including six patients with familial dysautonomia.RESULTS:All attempted operations were completed successfully laparoscopically, with only a few postoperative complications and acceptable short-term results. The clinical considerations and technical aspects unique to this specific group of patients are discussed.CONCLUSION:Laparoscopic Nissen fundoplication is feasible, safe, and effective, even in very small babies.

An 11‐year‐old child was evaluated for chronic gastric obstruction with intermittent symptoms from the age of 11 months. An upper gastrointestinal series performed at the age of 11 months was interpreted as compatible with severe pyloric stenosis due to a duodenal ulcer, but no surgery was recommended. Several other UGIT series, and at least four endoscopies performed during the following years by experienced gastroenterologists in Israel and in the United States, failed to reveal the true nature of the gastric outlet obstruction. The patient was operated upon shortly after referral to our institute. Upon celiotomy, a congenital antral web was found, the excision of which resulted in long‐term relief of all symptoms. The differential diagnoses of childhood gastric outlet obstruction and the possible pitfalls in the diagnostic workup are discussed.

Short term results following laparoscopic Nissen fundoplication were evaluated in 31 patients with symptomatic gastroesophageal reflux. 6 were females and 26 males, and they ranged in age from 5 months to 64 years (mean: 4.9 years in 19 younger than 18 years, and 39.3 years in 12 adults). Most of the adults who complained of pain and heartburn underwent pH monitoring, endoscopy, and manometry as needed. Milk scan was the most useful diagnostic tool for the evaluation of the children, who suffered mainly from gastroesophageal-related pulmonary disease. Indications for laparoscopic operation were identical with those for conventional open Nissen fundoplication. 1 case of dysautonomia died postoperatively; the rate of complications, mostly minor, was 22.5%. 3 patients required conversion to open Nissen fundoplication due to cardiorespiratory instability secondary to pneumothorax in 2, and to esophageal perforation in the third. 5 adults developed temporary dysphagia. 3 children had only partial improvement in their pulmonary disease following the operation, while the other 15 had complete relief. The total time for the laparoscopic operation averaged 245 minutes in adults, and 228 in children. Discharge was usually on the fourth postoperative day in adults (mean: 6.0 days). Regurgitation and heartburn were cured in 10 out of 11 adults (91%). All parents of children were satisfied. Symptomatic outcomes following laparoscopic Nissen fundoplication compare favorably with those of open surgery with respect to mortality, complications, and outcome.

A case of late‐appearing congenital diaphragmatic hernia in a premature infant with previously normal chest X‐ray is reported. Pleural effusion accumulation and resolution preceded herniation of the liver to the right hemithorax and development of respiratory symptoms. Chest X‐ray, ultrasound and computed tomography of the chest were useful in establishing the correct diagnosis. Pleural effusion without obvious cause should alert the paediatrician to the possibility of this rare condition.

OBJECTIVE:To evaluate whether cooling of ischemic intestine before and immediately after the release of the obstruction is more advantageous than is warming in an isolated ileal loop rat model.DESIGN:Prospective randomized experimental trials.SETTING:Laboratory.MATERIALS:Male Sabra rats.INTERVENTIONS:An isolated loop of ileum was created in rats and constantly perfused with warmed normal saline (37 degrees C). Intestinal ischemia was caused by clamping of the superior mesenteric artery for 15 minutes. Cooling of the isolated ileal loop to 32 degrees C by the application of cold saline was done for the last half of the ischemic period and continued during the first 10 minutes of reperfusion. The intestine was warmed to 37 degrees C for the next 20 minutes of reperfusion.MAIN OUTCOME MEASURES:The chromium 51-labeled ethylenediaminetetraacetic acid (51Cr-EDTA) plasma-to-lumen clearance rate was used for the evaluation of intestinal mucosal injury. Multiple intestinal samples were harvested at the end of the reperfusion period, and the histological features were evaluated.RESULTS:The 51Cr-EDTA plasma-to-lumen clearance rates were significantly lower (P < .001) in the rats in which cooling of the ischemic intestine was performed in comparison with the control group, indicating reduced intestinal mucosal injury in all three 10-minute reperfusion periods. Cooling also significantly changed the resultant histological damage to the intestinal mucosa exerted by ischemia (P < .001).CONCLUSION:Cooling of the ischemic intestine before the release of the obstruction and for a short period during reperfusion provides significant protection from ischemia/ reperfusion injury compared with the traditional method of warming the ischemic intestine.

Vascular tumors of the female genital tract are uncommon, and only a few cases have been reported in the ovary. We describe herein, an unusual tumor of the ovary: infantile hemangioendothelioma (cellular hemangioma of infancy) in a newborn. The tumor consisted of well-formed blood vessels and proliferating endothelial cells that were arranged in solid cordlike structures. The tumor permeated the ovarian stroma and entrapped normal ovarian follicles. By immunohistochemistry the neoplastic cells expressed factor VIII, CD34, and alpha smooth-muscle actin, and ultrastructurally they had the features of endothelial cells that were focally associated with pericytes. We examined simple sequence repeat (SSR) polymorphic markers in the tumor tissue, as well as in the patient's and parents' blood. The informative SSR markers were found to be identical in the tumor and in the patient's somatic cells. We suggest that the tumor described herein is a congenital infantile hemangioendothelioma arising from ovarian parenchymal cells rather than a teratoma originating from germ cells. A similar morphologic lesion has been described recently in the ovary and interpreted as monodermal teratoma composed of vascular tissue.

There is a large body of evidence that neutrophils may play an important role in the mucosal injury that follows ischemia of the intestine. Pentoxifylline (PTF), a methylxanthine derivative, prevents leukocyte adherence to vascular endothelium and restores intestinal blood flow following hemorrhagic shock and sepsis. The purpose of this study was to evaluate the protective properties of PTF in an ischemia-reperfusion model of the intestine and whether its action is mediated through tissue neutrophils as assessed by myeloperoxidase (MPO) determination. Intestinal ischemia of either 1 or 2 h was induced in rats by clamping the superior mesenteric artery, followed by a 17-min reperfusion period. PTF (25 mg/kg) or saline solution was injected IP 10 min prior to ischemia. Multiple bowel samples were harvested at the end of the reperfusion period and evaluated for histology and tissue MPO. PTF significantly changed the resultant histologic damage to the intestinal mucosa exerted by prolonged ischemia of 1 and 2 h duration, although the beneficial effect of PTF in this animal model was independent of the number of tissue neutrophils as assessed by tissue MPO levels. Pretreatment with PTF can thus reduce the histologic damage caused by prolonged ischemia to the intestine.

Children with familial dysautonomia (FD) often require an antireflux operation and gastrostomy to prevent the detrimental effects of aspirated gastric juice on the lungs and to facilitate liquid feeding. The aim of this study was to examine whether a laparoscopic procedure in such patients is as safe and effective as the traditional open technique. The data for all pediatric patients who underwent a laparoscopic antireflux procedure for familial dysautonomia were reviewed and compared with those the last pediatric patients with FD who were operated upon using the open technique, before the introduction of the laparoscopic procedure. Of the 61 children who underwent an antireflux procedure for FD (1978-1996), 13 were operated on laparoscopically. The authors found that the postoperative course of these patients was less complicated than that of patients who had the traditional laparotomy procedure. There was no need for mechanical ventilation during the postoperative course, and there were no respiratory complications. The mean hospitalization period was significantly shorter (7.9 days v 13.2 days). There were no complications attributable to laparoscopy, and the antireflux procedure has been effective in all patients (short-term follow-up). The authors conclude that laparoscopic procedures that include a modified Nissen fundoplication, gastrostomy, and appendectomy are feasible and as safe as conventional surgery for the treatment of FD in children. It appears that this approach has fewer complications than laparotomy, might reduce the need for postoperative mechanical ventilation, and is associated with a shorter postoperative stay.

The Antley-Bixler syndrome (ABS) is characterized by craniofacial, skeletal and urogenital anomalies. While most patients with ABS die of severe respiratory complications in their first months, long-term survivors have been reported. We report an infant girl, born to a consanguineous couple, with craniofacial and skeletal anomalies, consistent with ABS, in addition to atresia of the esophagus and trisomy 21.